Essentials-of-Oral-Pathology-3rd-Edition by purkait

The periodontium consists of several tissues extent and severity of disease increase with age.
namely the gingiva, the periodontal ligaments, This does not imply that all gingivitis will
the cementum and the alveolar bone, etc. The eventually progress to periodontitis. Since the
periodontal diseases are a group of heterogenous, adoption of the Community Periodontal Index
chronic destructive inflammatory diseases of the of Treatment Needs (CPITN), epidemiological
periodontium. data from various parts of the world can be
compared. This has confirmed that virtually all
EPIDEMIOLOGY adult individuals show some evidence of early
periodontitis but the advance disease affects only
Although there is evidence that at least, clinically, about 10-15 percent of the population. Tooth loss
several distinct types of chronic destructive perio- as a result of periodontal destruction is uncom-
dontal diseases may exist. The term gingivitis is mon before the age of 50 years unless the patient
used to designate inflammatory lesions that are has some other systemic diseases.
confined to the marginal gingiva. Once the
lesions extend to include destruction of the THE ROLE OF BACTERIA AND DENTAL
connective tissue attachments of the tooth and PLAQUE IN PERIODONTAL DISEASE
loss of alveolar bone, the disease is designated
periodontitis. • Epidemiological studies conducted by diffe-
rent investigators in many parts of the world
Chronic inflammatory periodontal diseases of suggest that there is a strong relationship bet-
varying severity affect practically all dentate ween the periodontal disease and the dental
individuals. Gingivitis is common in children, plaque.
even by the age of 3 years, and early periodontitis
may be detected in teenagers; in general, the • Clinical trials in humans and animals clearly
indicate that lack of maintenance of proper
Etiological factors of periodontal diseases oral hygiene causes accumulation of plaque,
which results in the initiation of gingivitis and
Local Factors • Microorganisms restoration of the proper plaque control
• Calculus measures rapidly prevents the disease and
• Food impaction brings back the gingival health.
• Faulty restorations
• Tooth malposition Important microorganisms in periodontal disease
• Mouth breathing habit
• Use of different drugs or • Actinobacillus actinomycetemcomitans

chemicals. • Actinomyces viscosus

Systemic Factors • Nutritional deficiency • Capnocytophaga group
• Pregnancy
• Diabetes • Eikenella corrodens
• Allergy
• Heredity • Fusobacterium nucleatum
• Immunological disorders
• Psychogenic factors • Porphyromonas gingivalis

• Prevotella intermedia

• Treponema

• Wolinella

Periodontal Diseases 529

• Administration of different antimicrobial CALCULUS
agents are proved to prevent the gingivitis
and this again establishes the role of plaque Sometimes, the dental plaque may become
bacteria in the causation of periodontal mineralized due to the deposition of calcium and
disease. other ions (which are coming either from the
saliva or the blood serum) and this mineralized
• Bacteria isolated from human dental plaque plaque is known as calculus.
are capable of inducing periodontal disease
when introduced into the mouths of gnoto- The calculus may also contribute to the
biotic animals. development of periodontal diseases, either by
harboring the plaque bacteria or by causing
• Several species of pathogenic bacteria have irritation to the gingival tissues.
been isolated from periodontal pockets that
have the capacity to invade tissue and evoke Factors causing accumulation and
destructive inflammatory changes. stagnation of plaque

• Healthy periodontal tissues of humans are • Calculus
associated with a scanty flora located almost • Food impaction
entirely supragingivally on the tooth surface. • Overhanging restoration
The microbial accumulations are 1 to 20 cells • Malocclusion
in thickness and comprise mainly gram- • Deep pockets
positive bacteria Streptococcus and • Mouth breathing
Actinomyces species predominate, for example
S. sanguis, A. naeslundii, A. viscosus, etc. OTHER LOCAL FACTORS

• In developing gingivitis, the total mass of Local factors include the anatomy of teeth,
plaque is increased and the microbial cell gingiva, and alveolar bone, alignment and
layers often extend to 110 to 300 cells in thick- occlusal relationship of teeth, and proximal
ness. Members of the genus Actinomyces restorations, etc. and these factors may affect the
predominate, but there is a substantial accumulation and growth of plaque or interfere
increase in strict anaerobes and gram-negative with its removal.
organisms. In long-standing gingivitis, gram-
negative organisms may account for 35 to 40 THE ROLE OF SYSTEMIC FACTORS
percent of the flora. The majority are located
subgingivally. Although dental plaque is the essential
etiological agent in periodontal disease, there are
• Microbial examination of subgingival plaque many systemic factors, which may alter the host’s
in periodontitis has revealed a complex flora response to local irritants and, therefore, could
rich in gram-negative rods, motile forms, and influence the development and progression of
spirochetes. the lesion.

• Black-pigmented bacteroid group are the Several systemic factors have been associated
predominant cultivable organisms in most with an increased incidence and severity of
subjects and they have been reclassified as periodontal disease or with modifying the course
Porphyromonas or prevotella species, for of that disease.
example Porphyromonas gingivalis, prevotella
melaninogenica, and prevotella intermedia, Diabetes Mellitus
etc.
• The relationship between diabetes and perio-
• The development of gingivitis and periodon- dontal disease is controversial. It is commonly
titis is associated with sequential colonization held that periodontal disease is more severe
and a progressively more complex flora, and progresses faster in diabetics than in
characterized by an increase in anaerobic, non-diabetics.
gram-negative and motile organisms rather
than a mere increase in the amount of plaque.

530 Essentials of Oral Pathology

• Vascular changes and defects in cellular • Other oral signs of the disease can be related to
defence mechanisms have been suggested as the associated pancytopenia and include muco-
possible mechanisms in which diabetes could sal pallor, necrotizing ulceration (particularly of
increase the susceptibility of periodontal tissue the oropharynx) petechial hemorrhages,
to irritants from dental plaque. gingival bleeding, and gingival ulceration, etc.

Pregnancy and Sex Hormones • Candidiasis and recurrences of herpetic
infection are also common.
• Severity of a pre-existing gingivitis increases
in pregnancy from the second to the eight • Alveolar bone loss and severe periodontal
month of gestation and then decreases. destruction have also been reported which, in
some patients, are caused by leukemic cell
• Increased level of circulating hormones are infiltration.
thought to be responsible, but the fact that so-
called pregnancy gingivitis can be resolved by Drugs
adequate plaque control emphasizes that the
hormonal changes simply modify the tissue With the exception of phenytoin, cyclosporine,
response to dental plaque and do not cause and nifedipine, which are associated with
the gingivitis themselves. Increased levels of generalized gingival hyperplasia.
sex hormones or their metabolites are found
in inflamed gingiva. Drugs which affect inflammatory and
immune responses, such as immunosuppresents
• The aggravation of gingivitis during preg- and non-steroidal antiinflammatory agents,
nancy is related mainly to progesterone, which might be expected to influence the course of
affects the function and permeability of the periodontal diseases, by modifying the response
gingivial microvasculature. of the host to product from microbial plaque. The
common drugs, which can affect the periodontal
• Localized gingivial hyperplasia also occurs du- tissue include, azathioprine, cyclosporine,
ring pregnancy (pregnancy epulis). Increased naproxen sodium, nifedipine, verapamil,
levels of gingivitis occurring around puberty estrogen and progesterone, etc.
and in some women taking oral contraceptives
may also be related to the concentration of Acquired Immunodeficiency Syndrome
circulating sex hormones. (AIDS)

Nutrition Several epidemiological studies indicate that the
periodontal status of many HIV-positive patients
• Nutritional factors can modify the course of, is similar to that of the general population.
but not initiate periodontal diseases. However, severe and atypical forms of perio-
dontal diseases may be seen in some patients,
• In human, the relationship between nutrition particularly those with AIDS.
and periodontal disease is controversial except
in rare cases of gross deficiency states. Smoking

• Advanced periodontal disease has been There is ample evidence that tobacco smoking is
reported in rural Nigerians who eat a protein– an important risk factor for the development and
deficient diet and in such children this is an progression of periodontal diseases. Smoking
important factor predisposing to cancrum oris. probably impairs the phagocytic function of
Polymorphonuclear neutrophils (PMN).
• Severe and prolonged deficiency of vitamin C
causes scurvy, which may be associated with PATHOGENESIS OF PERIODONTAL
haemorrhagic gingivitis and generalized DISEASE
edematous enlargement of the gingiva.
Host-parasite relationship—In healthy condition a
Blood Diseases balance exits between the challenge to the tissue
from microorganisms in dental plaque and the
• Acute leukemia may be accompanied by a host defense mechanisms. Disturbances in this
generalized enlargement of the gingivia
mainly due to infiltration and packing of the
tissues by leukemic cells.

Periodontal Diseases 531

host-parasite relationship lead to the develop- • It is essentially an acute inflammatory res-
ment of periodontal disease, but the transition ponse and within the inflammed area the
from healthy condition to gingivitis is not vessels become dilated, accompanied by the
precisely identifiable. formation of both a fluid and cellular exudates.

Host-parasite relationship in periodontal disease • Large numbers of predominantly polymorpho-
nuclear neutrophilic leucocytes (PMN)
Microbial plaque Host defences extravasate from the vessels and migrate
through the connective tissue to infiltrate the
Direct injury Salivary factors intercellular spaces of the junctional epithe-
Toxic products Crevicular fluid lium. They continue to migrate coronally and
Enzymes Epithelial barrier eventually enter the oral cavity through the
Antigenic challenge Transmigrating of base of sulcus.
neutrophils
immune response • This transmigration of the junctional epithe-
lium by PMN is a response to chemotactic
POTENTIAL FOR TISSUE REGENERATION stimuli released into and elaborated within the
sulcular area.
AND REPAIR
• The transmigration of PMN causes disruption
The host may be able to adapt to the imbalance of the junctional epithelium and widening of
in the relationship so that a new equilibrium is the intercelluar spaces, which impairs the
established and the disease may become arrested barrier functions of this tissue.
and remain stable over long periods of time.
Transient imbalances in the host-parasite • Exudation of protein–rich fluid, due to
relationship are likely to occur frequently and increased vascular permeability, accompanies
yet the natural history of periodontal disease in the cellular exudate. As the disease develops
humans usually spans decades, suggesting that and the volume of fluid-exudate contains all
equilibrium is rapidly restored and that for most classes of plasma proteins, notably immuno-
of the time destruction is not continuous but is globulins and complement, which may also
episodic in nature. play a role in controlling the initial bacterial
challenges.
The etiopathogenesis of periodontal disease
following withdrawal of oral hygiene procedures The Early Lesion
and the accumulation of dental plaque has been
described as occurring in four stages: • The early lesion develops within 4 to 7 days
following the onset of plaque accumulation
Stages of periodontal disease and overlaps with and evolves from the initial
lesion with no clear-cut dividing line.
• The initial lesion Gingivitis
• The early lesion • It develops at the site of the initial lesion, but
• The established lesion as it evolves, the area of inflammed gingival
connective tissue expands laterally and
• The advanced lesion Periodontitis apically, particularly as a narrow band
beneath the junctional epithelium, extending
The Initial Lesion towards the cemento-enamel junction.

• The initial lesion of gingivitis develops within Clinical Features of Gingivitis
2 to 4 days following the onset of plaque
accumulation. The changes are histological • Gingival tissue will become red or bluish red
and cannot be detected clinically. in color.

• The lesion is localized to the tissue around the • It becomes swollen, which may be either
base of the histological sulcus and involves localized or generalized in nature.
portions of the junctional and sulcular
epithelium and a microscopic area of • The gingiva has a boggy or spongy consis-
underlying gingival connective tissue. tency.

532 Essentials of Oral Pathology

• Bleeding occurs either spontaneously or by • Although, damage to fibroblasts in the
slight provocation (e.g. tooth brushing) from inflammed area would result in decreased
gingiva. synthesis, increased degradation involving
enhanced enzyme activity is the major
• The gingival attachment level on the tooth factor.
surface remains unchanged.
• The destruction of the extracellular matrix
• Radiographic changes in the bone are absent, may involve the activity of bacterial enzymes
as it is a superficial lesion. from the plaque, but is effected mainly by a
family of enzymes, the metalloproteinases
The Established Lesion derived from host cells.

• The established lesion of gingivitis develops • These enzymes principally collagenase and
within 2 to 3 weeks of the onset of plaque accu- stromalysin, are produced mainly by fibro-
mulation and evolves from the early lesion. blast, macrophages, and neutrophil leuco-
cytes.
• Histologically, there is accentuation of the
features of the initial lesion, but these changes • In periodontal disease, the release of metallo-
are confined to a narrow band beneath the proteinases is increased due to activation of
junctional and pathologically altered host cells by a variety of cytokines , especially
epithelium. interleukin-1, generated in the inflammatory
response to microbial plaque.
• There is further deepening of the gingival sul-
cus and further growth of subgingival plaque. The Advanced Lesion–Chronic Periodontitis

• The characteristic feature of the established • The advanced lesion corresponds to chronic
lesion, which distinguishes it from the early periodontitis, a disease characterized by des-
lesion, is the shift in the inflammatory cell truction of the connective tissue attachment
population within the infiltrated gingival of the root of the tooth, loss of alveolar bone,
connective tissue from predominantly and pocket formation (Figs 18.1 and 18.2).
lymphocytic (T cell) to predominantly plasma
cell (B cell) type. • Gingivitis in some patients may remain stable
for years, in other, it either progresses slowly
• Large amounts of immunoglobulins are to periodontitis or, in a minority of patients,
present throughout the connective and there may be rapid progression and advanced
epithelial tissue. bone loss occurring at an early age.

• There is continuing loss of collagen as the area • The earliest histological evidence of the
of inflammation slowly expands. progression of gingivitis to periodontitis is the
extension of inflammation beneath the base
• In areas away from the zone of destruction,
there may be varying attempts at repair
characterized by the formation of fibrous tissue.

Degradation of Extracellular Matrix Fig. 18.1: Photomicrograph of periodontal pocket

• Degradation of the extracellular matrix,
especially collagen, in periodontal disease is
of major importance, since destruction of the
connective tissue attachment results
ultimately in loss of the tooth.

• In health, normal collagen turnover is
reflected in a balance between the relative
rates of synthesis and degradation.

• The loss in disease could, therefore, be the
consequence of a decreased rate of synthesis,
increased rate of degradation, or a combi-
nation of both.

Periodontal Diseases 533

– supra-alveolar connective tissue
– alveolar bone
– periodontal ligament
• Predominance of plasma cells among the
inflammatory infiltrates.
• Loss of connective tissue attachment into:
– destruction of collagen
– apical migration of junctional epithelium
– pocket formation
• Destruction of alveolar bone
• Periods of quiescence/stability; episode of
destruction
– attempts at healing

Fig.18.2: Photomicrograph of advance periodontitis Clinical classification of periodontitis
with furcation involvement
Prepubertal A rare form affecting the deci-
of the junctional epithelium into the supra- periodontitis duous dentition, that may be
alveolar connective tissue. localized or generalized. Genetic
• Plasma cells dominate the infiltrate at all factors and a variety of medical
stages of the advanced lesions, although conditions may be associated.
lymphocytes and macrophages are also
present. Juvenile An uncommon form with onset in
• As the area and density of the infiltrate periodontitis puberty and adolescence and
increases there is destruction of collagen in the relativity well-defined clinical
supra-alveolar connective tissue and the fibers features.
loose their attachment to cementum.
• This is accompanied by apical migration of the Rapidly An uncommon form with onset in
junctional epithelium to cover the denuded progressive late adolescence and early adult-
root surface resulting in early true pocket periodontitis hood, characterized by episodes
formation. of localized or generalized perio-
• As the disease extends into the supporting dontal destruction. Many cases
tissue, there is progressive destruction of the have associated with defects in
fibers of the periodontal ligament accom- leukocyte function.
panied by osteoclastic resorption of the
alveolar bone. Adult type The most common form of perio-
• The junctional epithelium continues to periodontitis dontitis typically seen in adults
migrate apically, resulting in progressive over the age of 30 years.
deepening of the pockets.
• As the junctional epithelium migrates apically, CLINICAL FEATURES OF PERIODONTITIS
the more coronal and inflamed tissue loses its
specialized attachment and is converted into Prepubertal Periodontitis
pocket epithelium.
• Prepubertal periodontitis is a rare form of
PATHOLOGICAL FEATURES OF periodontitis presenting in childhood and
ESTABLISHED PERIODONTITIS involving the deciduous, and subsequently,
the permanent dentition.
• Persistence of the features of established
gingivitis • It is characterized by extensive destruction of
alveolar bone and is associated with a variety
• Apical extension of destructive inflamma- of uncommon systemic disease.
tion into:
• Most involved abnormalities in number and/
or function of PMN. Because of the deficient
or defective neutrophils such patients are
prone to severe systemic infections.

534 Essentials of Oral Pathology

Conditions causing widening of periodontal • The etiology and pathogenesis of the condition
ligament space remain obscure.

• Periapical abscess • The lesions are inflammatory and bacterial
• Current orthodontic therapy plaque is the prime etiological factor, but the
• Trauma from occlusion degree of destruction is not commensurate
• Scleroderma with the generally small amounts of plaque
• Osteosarcoma of the jaw. present.

• The Papillon-Lefevre syndrome is characteri- • However, the subgingival flora in juvenile
zed by skin lesions of palmar-plantar hyper- periodontitis is dominated by gram-negative
keratosis and severe periodontal destruction anaerobic rods, particularly Actinobacillus
involving both the deciduous and permanent actinomycetemcomitans. Capnocytophaga species
dentitions. and Eikenella corrodens may also be of etiologi-
cal significance.
• It is probably transmitted as an autosomal
recessive trait, but the mechanisms underlying • In addition to a unique flora, host factors have
the oral changes are uncertain, although a also been implicated. In particular, a familial
neutrophil abnormality has been reported. pattern has been found in several cases and
there is increasing support for the suggestion
Juvenile Periodontitis that genetic factors are involved.

• Juvenile periodontitis is a rare form of • Abnormalities in cell-mediated immunity and
periodontitis, which commences in young in PMN function have also been demon-
people and is clinically distinct from the usual strated.
form of periodontitis seen in adults.
Rapidly Progressive Periodontitis
• It commences around puberty and there is a
higher incidence in females. • Less common than juvenile periodontitis,
rapidly progressive periodontitis has an onset
• The disease is characterized by rapid destruc- between puberty and 30 years of age but lacks
tion of alveolar bone with vertical bone loss well-defined characteristic.
resulting in deep infrabony pockets (Fig. 18.3).
• The periodontal lesions are severe and gene-
• Initially, the permanent first molar and/or ralized with evidence of rapid bone destruction
maxillary incisor teeth are affected, usually occurring within a few weeks or months.
symmetrically, but the number of teeth
involved increase with age. • The gingiva my appear actually inflamed or
relatively normal, and the amounts of
• The pattern of involvement of teeth tends to microbial deposits are highly variable.
follow their sequence of eruption.
• Almost the entire dentition is usually affected
and the disease may either progress without
remission to tooth loss or subside and become
quiescent.

• Vertical or angular bone loss occurs with
marked difference in attachment loss between
adjacent teeth or different surfaces of the same
tooth.

Fig.18.3: Photomicrograph of infrabony Adult Type Periodontitis
periodontal pocket
• Adult type periodontitis is by far the most
common form of chronic periodontal disease
and is characterized by its chronicity.

• The onset of disease is in early adult life (or
even before). But in the majority of patients

Periodontal Diseases 535

does not progress to tooth loss until after 50
years of age.
• A generally regular pattern of predominantly

horizontal bone loss is seen with suprabony
pocket formations.
• The entire dentition is usually involved, with
the lower incisors and molar tending to show
the maximum bone loss.

Factors affecting the prognosis of Fig. 18.4: Drug-induced gingival hyperplasia
periodontal diseases
Common drugs associated with gingival
• Oral hygiene status hyperplasia (Figs 18.4 and 18.5)
• Patient motivation
• Age of the patient Anticonvulsants
• Tooth factor • Phenytoin
• Host resistance • Carbamazepine
• Ethotoin
GINGIVAL HYPERPLASIA • Felbamate
• Mephenytoin
An increase in the number of cells causing tissue • Phenobarbital
growth is called hyperplasia while an increase • Sodium valproate
in the size of cells causing tissue growth is called Calcium channel blockers
hypertrophy. • Amlodipine
• Bepridil
Gingival hyperplasia refers to the excessive, • Diltiazem
exuberant proliferation of gingival tissue causing • Nifedipine
swelling or overgrowth of the gingiva. • Verapamil
Cyclosporine
Gingival hyperplasia may be of two types: Erythromycin
A. Inflammatory hyperplasia Oral contraceptives
B. Fibrous hyperplasia.
Gingival Hyperplasia Associated with
CAUSES OF GINGIVAL HYPERPLASIA Vitamin C Deficiency
Vitamin C helps in the synthesis of collagen by
Causes of inflammatory hyperplasia: helping in the hydroxylation of proline and
• Vitamin C deficiency (scurvy) lysine.
• Leukemias
• Chronic hyperplastic gingivitis Defective collagen synthesis in Vitamin C
• Endocrine imbalance (puberty/pregnancy) deficiency produces scurvy and the disease will
• Sarcoidosis have the following manifestations:
• Crohn’s disease. • The gingiva becomes tendered, edematous

Causes of fibrous hyperplasia: and swollen.
• Heredity (genetic) • It has a spongy consistency and bleeds
• Drug intake
• Orofacial angiomatosis frequently.
• Wegner’s granulomatosis
• Idiopathic.

536 Essentials of Oral Pathology

Fig. 18.5: Histopathology of drug-induced Fig. 18.6: Localized gingival hyperplasia
gingival hyperplasia
conditions are often associated with gingival
• The crest of the interdental papillae appear hyperplasia.
“red or purple”
Hormonal imbalance mostly increases the
• Ulceration and necrosis of the gingival tissue proliferative potential of the gingival tissue in
occurs commonly. response to the irritations caused by plaque
bacteria and local irritants.
• Gingival sulcus is often filled with blood clot
and there may be hemorrhage from any part Clinically the following manifestations will develop
of the body following slight trauma. • The gingiva becomes red, edematous and

• Foul smell is often present in the mouth. swollen.
Administration of Vitamin C and improve- • It may or may not be painful but bleeds

ment of the oral hygiene will cure the disease. frequently.
• Sometimes, a localized “tumor-like” growth
Gingival Hyperplasia Associated with
Leukemias may develop on the gingiva during pregnancy
and it is often known as “pregnancy tumor”
Acute monocytic, lymphocytic or myelocytic (Fig. 18.6).
leukemias often manifest intraorally by gingival • The condition regresses spontaneously after
hyperplasia, which occurs predominantly due to the pregnancy period is over.
the infiltration of malignant cells into the gingival No treatment is required except oral pro-
tissue. phylaxis and maintenance of oral hygiene.

Clinically the following manifestations develop: Gingival Hyperplasia Associated with
• The gingiva becomes soft, edematous and Crohn’s Disease

swollen The Crohn’s disease is characterized by granlo-
• It is usually painful and has a purplish, glossy matous superficial ulceration of the intestinal
epithelium, with frequent development of
appearance. multiple fistulas. The disease produces the
• Pallor in the surrounding mucosa with following oral manifestations.
• Granular, erythematous swelling of the
development of petechiae or ecchymosis is
often observed. gingiva.
• Ulcerations and severe hemorrhage often • Ulceration and occasional bleeding is also
occur in the gingiva. The diagnosis is confir-
med through hematological investigations. reported.
• “Cobble-stone” appearance of the buccal
Gingival Hyperplasia Associated with
Endocrine Imbalance mucosa with many linear hyperplastic folds
• Diffuse indurated swelling on the lips.
Abrupt hormonal changes often take place in the • Multiple ulcerations on the palate.
body during puberty or pregnancy, etc. and these

Periodontal Diseases 537

Fig.18.7: Dilantin hyperplasia of gingiva Fig. 18.8: Photomicrograph of dilantin hyperplasia

Gingival Hyperplasia Due to Dilantin Fig. 18.9: Gingival fibromatosis
Sodium Therapy
plasia and in such cases, the gingiva clinically
Dilantin sodium is an antiepileptic drug and use appears swollen and red. The enlargements may
of this drug may induce gingival hyperplasia (as cause false gingival pocket formation on few
its side effect) (Figs 18.7 to 18.9). occasions.

Clinically the following Manifestations Gingival Hyperplasia in Wegener’s
Develop Granulomatosis
Focal or diffuse gingival swelling can occur in
• The disease begins with painless enlargement Wegener’s granulomatosis and the lesion is
of the interdental papilla. characterized by epithelial proliferation and
dense inflammatory cell infiltration.
• The swelling is rough, lobulated and has a
pebbly surface. DESQUAMATIVE GINGIVITIS

• The gingival tissue is of normal color and often DEFINITION
there is an increase in stippling. Desquamative gingivitis refers to the condition
in which the gingival epithelium sloughs
• It is firm and dense, with no tendency to bleed. spontaneously or can be scrapped off with gentle
• The gingival growth ceases after the drug rubbing.

therapy is stopped.
Beside dilantin sodium, gingival hyperplasia
may also occur due to other drugs, e.g. nifedipine
and cyclosporine, etc.

Hereditary Gingival Hyperplasia

Hereditary or familial gingival hyperplasia occurs
among several members of the same family.

The gingiva is usually firm and resilient and
is of normal color. Pain and hemorrhage, etc. are
usually absent. In some patients, the gingival
growth is so severe that it may cover up the entire
crowns of the teeth and may even prevent the
eruption of teeth in younger individuals.

Gingival Hyperplasia in Orofacial
Angiomatosis

Angiomatous proliferation of the gingival blood
vessels may sometimes cause gingival hyper-

538 Essentials of Oral Pathology

Desquamative gingivitis is not a disease entity ACUTE NECROTIZING ULCERATIVE
but a clinical term applied to the gingival mani- GINGIVITIS (ANUG)
festation of several different diseases.
Acute necrotizing ulcerative gingivitis is a
CLINICAL FEATURES relatively rare condition and is characterized
clinically by severe necrosis of the free gingival
• Clinically gingival tissue appears smooth, red, margin, the crest of the gingiva and the interdental
and edematous with loss of stippling; and papillae, etc.
there are areas of superficial ulceration, ero-
sion or spontaneous desquamation of varying Acute necrotizing ulcerative periodontitis is the
severity. advanced lesion of the same ulcerative process
(ANUG) and occurs when the necrotizing
• Vesicles or bullae (filled with clear fluid or process causes loss of epithelial attachment and
blood), white flecks or striae may also be seen spreads into the deeper tissues of periodontium,
depending on the underlying etiology. leading to periodontitis

• Severe pain, burning sensation and difficulty ETIOLOGY
in food intake are the common complaints.
Acute necrotizing ulcerative gingivitis is a fuso-
• The disease has a gradual onset and it initially spirochetal disease and is caused predominantly
affects only limited part of the gingiva, by the fusiform bacilli and a spirochete called
however with time, the disease spreads to Borrelia vincentii.
involve larger areas of the gingival tissue.
Microorganisms often implicated in ANUG
• The involvement is patchy but the buccal and
labial gingivas are more commonly affected • Borrelia vincentii
than lingual or palatal tissues. • Fusobacterium fusiformis
• Prevotella intermedia
• The condition is more common in females (80 • Porphyromonas gingivalis
percent) than males and most cases occur after • Selenomonas sputigena
30 years of age. • Leptotrichia buccalis

• Mucous membrane pemphigoid and lichen Interestingly, it has been observed that, the
planus account for most cases of clinical disease affects multiple numbers of people
desquamatic gingivitis. staying under the same living conditions. This
often gives a false impression of the contagious
• Other cases my represent local hypersensi- nature of this disease.
tivity reactions to various substances, for
example tooth pastes, cosmetics. chewing- Precipitating factors in ANUG
gums, and cinnamon, etc.
• Immunosuppression (AIDS, infectious mono-
• The gingival reaction associated with nucleosis)
chewing-gum hypersensitivity (has also been
referred to as “plasma-cell gingivitis”) • Sudden change in lifestyle.
widespread distribution of large numbers of • Poor nutritional status.
plasma cells throughout the gingiva. • Poor oral hygiene.
• Lack of rest and sleep.
• Several other uncommon etiological factors • Local tissue damage local trauma.
have also been suggested, which include hor- • Recent debilitating diseases, (e.g. bacterial
monal disturbances in menopausal females,
unusual manifestations of chronic infection. infections, diabetes, blood dyscrasias, etc).
• Emotional and professional stress.
• Abnormal responses to dental plaque will • Down’s syndrome
exacerbate the inflammation and maintenance • Smoking.
of adequate oral hygiene is important in
management, but may be difficult because of
soreness and bleeding associated with
erosions.

Periodontal Diseases 539

CLINICAL FEATURES epithelial attachment the condition is called
‘necrotizing ulcerative periodontitis’.
• Acute necrotizing ulcerative gingivitis usually • Most of the patients develop systemic
occurs among young and middle aged adults, manifestations in the form of leukocytosis,
between the ages of 15 and 35 years and males tachycardia and GI disturbance, etc.
suffer more often than females. • When the necrotizing process of ANUG
extends further through the oral mucosa and
• Stressed professionals like army recruits tend reaches to the extraoral skin surface, the
to suffer more (7 percent) from the disease condition is called ‘noma’ or cancrum oris.
than the normal population (1 percent).
HISTOPATHOLOGY
• Moreover, young children suffering from
malnutrition are also more vulnerable to the • The affected gingival tissue shows inflam-
disease. mation, ulceration and extensive necrosis.

• Initially the gingiva becomes red, edematous, • The gingival stratified squamous epithelium
hemorrhagic and painful. is often replaced by a thick, fibrinopurulent
“pseudomembrane”.
• Later, on, a sharply demarcated “punched-
out” crater-like erosion of the interdental • The pseudomembrane usually consists of
papillae occurs. microorganisms, polymorphonuclear neutro-
phil (PMN), and necrotic tissue debris, etc.
• The gingiva is often covered by a gray
“pseudomembrane” with accumulation of • The unaffected areas of the gingival tissue
necrotic tissue debris. show a general lack of keratinization.

• Patient have pronounced spontaneous • The underlying connective tissue shows
bleeding tendency, exquisite pain and an intense hyperemia and inflammatory cell
extremely unpleasant fetid odor in the mouth. infiltration by PMN.

• Rarely, the gingival lesion may extend to the DIFFERENTIAL DIAGNOSIS
mucosal surfaces of soft plate and tonsils; and
thereby resulting in the condition called • Primary acute herpetic gingivostomatitis
necrotizing ulcerative stomatitis. • Erosive lichen planus
• Cicatricial pemphigoid
• Patients often develop headache, fever, • Drug allergy.
malaise and lymphadenopathy, etc.
TREATMENT
• Often there is difficulty in taking food due to
increased salivation and a metallic taste in the • Local debridement of necrotic tissue with
mouth. H2O2.

• When the necrotizing process leads to the • Administration of metronidazole, tetracycline
development of periodontiris with loss of and penicillin, etc.

Key points of ANUG • Oral hygiene motivation.

• Predominantly affects young adult males LATERAL PERIODONTAL ABSCESS

• People with smoking habits and history of minor DEFINITION

respiratory infections are more prone. The lateral periodontal abscess is a localized area
of suppurative inflammation arising within the
• Punched-out ulcers occur at the tip of interdental periodontal tissue alongside a tooth and is
distinct from the more common periapical
papilla which spreads along the gingival margins. abscess.

• Gingival bleeding, soreness and extreme foul

breath.

• The disease is caused by spirochaetes and

fusiform bacilli.

• No significant fever, lymphadenopathy or

systemic complications

• Responds well to metronidazole and good oral

hygiene care.

540 Essentials of Oral Pathology

ETIOLOGY • Discharge of pus relieves the acute symptoms
and the lesion may heal or become chronic
Most lesions arise in patients with pre-existing with intermittent discharges.
advanced periodontitis and it may occur either
as a direct result of an increase in virulence and • The chronic abscess may be asymptomatic or
toxic factors released by plaque organisms or give rise to episodes of dull pain. Acute
secondary to reduction in host resistance. exacerbations are common.
• Obstruction to the drainage of exudate from
RADIOGRAPHIC FEATURES
a pocket predispose to abscess formation. This • Radiographic appearances are very variable
may occur particularly in infrabony pockets
pursuing a tortuous course around the root, and are influenced by the extent of previously
or where fibrosis or edema in the superficial existing bone destruction, the stage of the
parts of the pocket causes tight approximation abscess, and its location.
of the soft-tissue wall to the neck of the • There is often extensive pocketing and abscess
tooth. formation may be accompanied by rapid
• Impaction of foreign material, such as food deepening of such defects, but in the early
debris, into a pocket may also lead to abscess stages of an acute lesion there may be no
formation. associated radiographic changes.
• Following a traumatic injury to a tooth • In deep-seated lesions, there may be a discrete
• Lateral perforation of the root in endodontic radiolucent area along the lateral aspect of the
therapy root.
• Stab infections, which arise when a foreign
object, such as a toothbrush bristle or fish bone, TREATMENT
penetrates the tissue introducing infection into • Drainage
the periodontal ligament. • Antibiotics
• Elimination of the etiologic factors
CLINICAL FEATURES • Surgical intervention.

• Clinically, periodontal abscess may be acute PERICORONITIS
or chronic.
DEFINITION
• An acute abscess develops rapidly and is
accompanied by throbbing pain, redness, Pericoronitis is inflammation of the soft tissue
swelling, and tenderness of the overlying overlying the crown of an impacted or partially
mucosa. erupted tooth; and is seen commonly in asso-
ciation with mandibular third molars.
• The affected tooth is extremely sensitive to
palpation, tendered to percussion but it is CAUSES
mostly vital. Pericoronitis mostly occurs due to accumulation
of bacterial plaque and food debris in the space
• There can be sensitivity, mobility and/or between the crown of the impacted or partially
extrusion of the adjacent teeth. impacted tooth and the overlying gum flap;
leading to inflammation. Other factors which
• The abscess may discharge spontaneously may aggravate the condition include –opposing
through the mouth of a pocket, but in deep- tooth biting on the gum overlying the impacted
seated lesions or where drainage is obstructed, tooth and acute ulcerative gingivitis, etc.
it may track and present with a sinus opening
on the mucosa somewhere along the length CLINICAL FEATURES
of the root. • Inflammatory edema in the pericoronal tissue

• Foul taste in the mouth is almost always leads to swelling and pain in the gum flap
present. around the tooth.

• The associated general symptoms include
fever, malaise and lymphadenopathy, etc.

Periodontal Diseases 541

• The swollen pericoronal tissue predisposes to Black-Gray Stains
further trauma from the opposing teeth and These staining mostly occur due to amalgam
this leads to exacerbation of the pain and restorations and are more intense in young indi-
inflammation. viduals as they have more open dentinal tubules.

• The usual symptoms of pericoronitis include Brown or Black Stains
pain, swelling, erythema, tenderness in the These stains are produced by sulphides or silver
gum flap and a bad taste; it is associated with nitrates. Brown and black stains appear either
occasional oozing of pus from beneath the flap. as a thin line along the gingival margin or as wide
band on the tooth surface. These stains are
• Because of the pain and swelling it is difficult commonly seen on the teeth located adjacent to
to close the jaws. the salivary gland duct orifices.

• The pain is generally very severe, which often Brown Stains
radiates to the ear and floor of the mouth. Tobacco, tea and coffee, etc often cause brown
discoloration of teeth; Smokers do have
• Limitation of mouth opening due to trismus discoloration of lingual surfaces of mandibular
and discomfort on swallowing may be incisors.
frequently present.
Green Stains
• Recurrent inflammation can result in severe Green stains are generally produced by metals,
localized ANUG-like necrosis of the tissue. e. g. copper and nickel, etc and these stains are
usually seen on the labial surface of upper
• In severe cases, an acute pericoronal abscess anterior teeth as a band and are probably caused
may develop, which can either remain loca- by the blood pigments secondary to gingival
lized or spread diffusely leading to cellulitis hemorrhage. Green stains are produced due to
and space infections. excessive consumption of chlorophil containing
foods.
• In some cases, the inflammation in the
pericoronal tissue may be chronic in nature; in Orange or Yellow Stains
which the clinical symptoms are very little. Orange or yellow stains are formed on the
gingival third of the teeth and are easily removed.
TREATMENT
Treatment is done by antibiotic therapy, (B) INTRINSIC STAINS OF TOOTH
elimination of obstruction in the path of eruption
of the involved tooth or extraction of the tooth Causes of intrinsic stain Color change in tooth
whenever necessary.
Aging Yellow-brown
STAINING OF TEETH
Death of pulp Gray-black
(A) EXTRINSIC STAINS OF TOOTH (non-vital tooth)

Causes Fluorosis White-yellow or
• Tobacco yellow-brown
• Coffee, tea and cold drinks, etc.
• Chromogenic microorganisms Tetracycline Yellow-brown
• Mouth washes, e. g. chlorhexidiene gluconate
Minocycline Blue-gray
Mostly the brown, black, green or orange
stains are produced by the chromogenic bacteria Internal resorption Pink
and these occur commonly on the labial surfaces
of maxillary anteriors in children. Dentinogenesis imperfecta Blue-gray

Common Stains in Teeth Amelogenesis imperfecta Yellow-brown
Yellow-Brown Stains
Chlorhexidine produces yellow-brown discolo- Congenital porphyria Yellow or brown-red
ration, especially on the proximal surfaces of
teeth near the cervical region. Erythroblastosis fetalis Yellow-green

Jaundice Yellow-green

Lepromatous leprosy Pink or red

542 Essentials of Oral Pathology

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pathogenesis of gingival overgrowth. Periodontology
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1986;21:221-32. Palcanis KG, Ranney RR. Distribution of
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Kenney (eds): prevention of Periodontal Disease
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IgG subclasses in human periodontal disease I
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human periodontal diseases. Journal of Periodontology lymphocytes and plasma cells J periodont Res
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8. Brown RS, Beaver WT, Bottomley. On the mechanism 24. Manson JD. Juvenile periodontitis (periodontosis). Int
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and Maxillofacial Pathology Saunders, an imprint of
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Characterization of calculus matrix calcification
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Periodontal status of patients with abnormalities of the
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periodontopathic microorganisms and their oral
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periodontitis. International Dental Journal Socransky, SS. A study of the bacteria associated with
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Periodontol 1979;6:278.
16. Genco RJ, Slots J. Host responses in periodontal
diseases. Journal of Dental Research 1984;63:441-51. 33. Van Palenstein Helderman WH. Microbial etiology of
periodontal disease. J Clin Periodontol 1981;8:261.

A large number of dermatological disorders may

present with some oral lesions, either as a part
of their manifestations or as the sole feature of
disease. Most of these disorders have a diverse
etiopathogenicity and, more importantly, few of
them are potentially malignant in nature.

Diagnosis of dermal lesions usually depends
on their oral and cutaneous manifestations,
histopathology and immunohistochemistry, etc.

The features of some of the important derma-
tological diseases will be briefly discussed in this
chapter.

HEREDITARY ECTODERMAL Fig. 19.1: Hereditary ectodermal dysplasia
DYSPLASIA (typical facial profile)

Hereditary ectodermal dysplasia is an inherited • There is fine wrinkling and hyperpigmenta-
X-linked recessive disorder, characterized by the tions over the periocular skin.
defective formation of ectodermal structures of
the body (e.g. skin, teeth, nails, sweat glands, • Patients have malformed finger nails which
sebaceous glands and hair follicles). are often brittle.

CLINICAL FEATURES (FIGS 19.1 TO 19.3) • Complete or partial anodontia occurs that
involves both deciduous as well as the
• The three most outstanding features of permanent dentition (only the canines are
ectodermal dysplasia are hypohydrosis (lack often present).
of sweating), hypotrichosis (absence of hair)
and hypodontia (absence of teeth). • The teeth which may be present are often small
and abnormal in shape; e.g. the incisors look
• The disease occurs more frequently among tapered, conical or pointed while the molars
males than females. look narrow and much smaller in diameter
(Fig. 19.2).
• The patients often have soft, dry and smooth
skin with little or no tendency for sweating; • Xerostomia is a constant feature which occurs
due to decreased number of sweat glands. due to the decreased salivary secretion.

• Patients may have an unexplained fever, as • The patient may also have rhinitis, sinusitis,
release of heat from body through sweating pharyngitis, etc. with dysphagia and
is not possible and moreover they cannot hoarsenoess of voice.
endure warm temperature.

• The hair over the scalp, eyelashes and eyebrows
are often fine, scanty and blond (Fig. 19.1).

• The sebaceous glands are also absent
(asteatosis).

544 Essentials of Oral Pathology

Fig. 19.2: Hereditary ectodermal dysplasia patient showing Key points of hereditary ectodermal dysplasia
formation of only few conical shaped teeth
• Ectodermal dysplasia is a hereditary disorder
characterized by defective formation of
ectodermal structures of the body, e.g. skin,
teeth, nails, sweat glands, sebaceous glands and
hair follicles.

• Hypohydrosis, hypotrichosis and hypodontia are
the three most outstanding features of
ectodermal dysplasia.

• Hypohydrosis or lack of sweating causes difficulty
in releasing the body heat through sweating and
the patients thus always have high body
temperature.

• There is complete or partial lack of development
of tooth and patients require artificial prosthesis
from young age.

• Xerostomia (dry mouth) is a constant feature
due to decreased salivary secretion.

• Patient have a typical facial appearance with
depressed nasal bridge, frontal bossing,
protuberant lips, etc.

Fig. 19.3: Hereditary ectodermal dysplasia X-ray showing PSORIASIS
almost complete lack of tooth formation in lower jaw
Psoriasis is a common self-limiting, chronic
• The patient frequently exhibits “pili torti” inflammatory dermatological disease of
(kinky hair), cleft palate and popliteal ptery- unknown etiology. Some investigators believe
gium. that its occurrence is genetically determined.
Psoriasis exhibits an increased proliferative
• Patient with ectodermal dysplasia always activity of skin and mucous membrane.
have a typical facial appearance characterized
by depressed nasal bridge, frontal bossing CLINICAL FEATURES
and protuberant lips, and because of this,
most of the patients are often mistaken for • The disease occurs predominantly in the
siblings. second and third decade of life. There is no
sex predilection.
TREATMENT
• Psoriasis clinically presents well-demercated,
The typical clinical manifestations always painless, dry, erythematuos patches with
confirm the diagnosis of this disease and there is silvery scale on the surface.
no specific treatment for it. Artificial dentures
(with soft liners) are constructed and are changed • These lesions often develop on the skin over
from time to time to cope up with the growth of the elbows, knees, scalp, chest, face, etc.
the jaws and artificial saliva is given to keep the
mouth moist. • The patches are well-circumscribed and may
be present for years on the skin, few lesions
can even produce sterile pustules.

• The lesions enlarge at the periphery and the
disease often shows periods of remissions and
exacerbations.

• These skin lesions are often symmetrically
distributed and these are more severe during

Oral Aspects of Dermatological Disorders 545

the winter and the condition is much better CLINICAL FEATURES
during the summer.
• The psoriatic lesions are mostly asymptomatic • The disease commonly occurs in children or
besides occasional complains of itching. young adults and it affects several people at a
• Mental anxiety or stress often increases the time.
severity of the disease.
• If the deep scales on the surface of the lesion • This disease often shows seasonal outbreaks
are removed, one or two tiny bleeding points and it is mostly noticed during spring or
are often disclosed and this phenomenon is autumn.
known as “Auspitz’s sigh”.
• Although rare, oral lesions of psoriasis present • Clinically, pityriasis rosea presents
erythematous patches with white scaly superficial, light red macules or papules over
surfaces over the lips, palate, gingiva, cheek, the skin, which cause slight itching.
etc.
• Some lesions in the oral cavity may appear as • A typical bright-red skin rash is present in the
well-defined, grayish-white or yellowish- affected area about 10 to 15 days prior to the
white papules. development of original macules or papules
• In some cases, these oral lesions may resemble and this primary skin rash is known as
“geographic tongue”. “herald patch”.
• Sometimes psoriasis may be accompanied by
arthritis and the condition is often called • Sometimes, the disease may be accompanied
“psoriatic arthritis” and it can affect the TM by low grade fever, headache, lymphadeno-
Joint as well. pathy, etc.

MICROSCOPIC FINDINGS • In the oral cavity pityriasis rosea develops
erythematous macules over the cheek,
• The oral epithelium shows atrophy with tongue, palate, etc. These macules often have
hyperparakeratosis, absence of granular cell a central grayish area of desquamation.
layer and elongation or clubbing of the rete-
pegs. • Oral mucosa in the affected areas also exhibits
ulceration, vesicle formation and hemorrhage,
• Intraepithelial microabscess formation etc.
(abscess of Munro) is an important histolo-
gical finding of psoriasis. HISTOPATHOLOGY

• There is always an increased mitotic activity • The oral epithelium in pityriasis rosea exhibits
seen in the psoriatic skin or mucous mem- hyperorthokeratosis or parakeratosis along
brane. with mild degree of acanthosis.

• The sub-epithelial connective tissue shows • Ulceration, hemorrhage and chronic inflam-
many dilated capillaries and perivascular matory cell infiltration are found in the basal
infiltrations of lymphocytes or histiocytes. epithelium as well as in the juxta-epithelial
connective tissue.
TREATMENT
TREATMENT
No treatment is required as the disease
undergoes spontaneous regression. Since, it is a self-limiting condition, no treatment
is usually necessary.

INCONTINENTIA PIGMENTI

It is a relatively rare, serious type of inherited
genodermatosis; which is transmitted as a sex-
linked dominant trait.

PITYRIASIS ROSEA CLINICAL FEATURES

Pityriasis rosea is a rare mucocutaneous disease, • The disease occurs mostly during infancy.
which probably develops as a result of some viral Although, it is more common among females,
infections. it is often lethal among males.

546 Essentials of Oral Pathology

• Clinical manifestation begins to appear shortly ERYTHEMA MULTIFORME
after birth and is characterized by slate-grey
pigmentation of the skin with formation of Erythema multiforme is an acute inflammatory
vesicles or bullae over the trunk and limbs. dermatological disorder characterized by
extensive blistering and ulcerations. The disease
• Lichenoid, papillary or verrucous lesions also frequently involves skin, mucous membrane and
develop in the skin. sometimes the internal organs.

• Oral mucosa exhibits patchy, plaque-like or ETIOLOGY
verrucous looking white lesions on the buccal
mucosa. Although the exact etiology of erythema
multiforme is obscure, some precipitating factors
• Patients may also have epilepsy, strabismus have been identified, which are shown below in
with nystagmus, partial anodontia, etc. the table.

• The teeth are small and cone shaped and the CLINICAL FEATURES
condition affects both deciduous as well the
permanent dentitions. • Erythema multiforme frequently occurs
between the age of 15 and 40 years and males
HISTOPATHOLOGY are affected more often than females.

• During the verrucous stage of the disease, • The prodromal symptoms occur about one
intra-epithelial vesicle formation is often seen week before the onset of the disease and these
with accumulation of large number of include fever, malaise, headache, cough, sore
eosinophils. throat, etc.

• Dermal or submucosal accumulations of • Soon after rapidly developing round erythe-
macrophages and melanin granuals are also matous macules, papules or vesicles appear
seen. over the skin.

• White areas display hyperorthokeratosis or • These lesions develop symmetrically over the
parakeratosis with acanthosis. hands and arms, legs and feet, face and neck,
etc.
• Individual cell keratinization is also some-
times seen. • In severe cases, large bullae may also develop
over the skin.
TREATMENT

No specific treatment is available.

Infections Precipitating factors in erythema multiforme

• Tuberculosis
• Herpes simplex (Type I and Type II) infections
• Mycoplasma pneumoniae
• Infectious mononucleosis
• Histoplasmosis.

Drug hypersensitivity • Barbiturates
• Sulfonamides
• Phenylbutazone
• Salicylates
• Oral pills

Hyperimmune reactions Results formation of antigen-antibody complex
against the sub-mucosal and dermal blood vessels.

Miscellaneous factor • Radiation therapy
• Crohn’s disease
• Vaccinations

Oral Aspects of Dermatological Disorders 547

Fig. 19.4: Erythema multiforme showing ulceration • The ulcers are diffuse, extremely painful, have
of the facial skin with erythematous change irregular borders and are normally covered
by a slough. These ulcers can be secondarily
• The classic dermal lesions of erythema infected in many cases.
multi-forme which often appear on the
extremities are called “target”, “iris” or • Patients often complain of foul smell in the
“bull’s eye”, etc. mouth and difficulty in eating or swallowing,
these often lead to severe weakness and
• The target lesions are red macules of one dehydration.
centimeter or more in diameter with a bluish
cyanotic center.They often consists of • The cutaneous and mucosal lesions may occur
concentric erythematous rings separated by either separately or simultaneously.
rings of near normal color on the skin.
• The entire disease process may recur in a cyclic
• Severe form of erythema multiforme may order at an interval of few months and the
cause widespread sloughing and ulceration of cycle may characteristically continue for one
the skin, and the mucous membrane of the to two years, with increasing in severity after
entire body (Fig. 19.4). every recurrence.

• Mucosal lesions in this disease also consist of • Patients with erythema multiforme may also
macules, papules or vesicles, etc. and these are develop tracheobronchial ulcerations and
mostly seen on the tongue, labial mucosa, pneumonia, etc.
buccal mucosa, floor of the mouth, etc.
• Erythema multiforme is a self limiting disease
• The oral mucosal lesions are sometimes more and it regresses spontaneously.
prominent than the skin lesions and
sometimes they are the only ones present. • The disease is uncommon among children and
older individuals, however, if it occurs in the
• The vesicles of the mucosal surfaces are often older people, the possibility of an internal
short-lived and they readily become eroded carcinoma should not be ruled out.
or ulcerated and bleed profusely.
Stevens-Johnson Syndrome
• However, new vesicles appear in the oral
mucosa in about 10 days time and the process It is a severe form of erythema multiforme
continues for nearly three to four weeks. (also known as erythema multiforme major)
that simultaneously involves the skin, eyes,
oral mucosa and genitalia. This condition is
often triggered by a drug and not by an
infection.

The clinical features of this condition are as
follows:

Skin Lesions

• Severe lesions of macules, papules, vesicles,
bullae, etc. (as discussed earlier).

Oral Mucosal Lesions

• Large vesicles or bullae often develop which
rupture and leave painful ulcers (Fig. 19.5).

• Pain and hemorrhage from the ulcers cause
difficulty in taking food.

• Ulceration and bloody encrustation are often
present on the lips.

• Mucosal lesions are self-limiting and complete
healing, usually occurs in about two to three
weeks time. Recurrence is common.

548 Essentials of Oral Pathology

Fig. 19.5: Erythema multiforme producing intensely Key points of erythema multiforme
erythematous ulcerations of the buccal mucosa
• Erythema multiforme is serious type of der-
Eye Lesions matological disorder characterized by extensive
blistering and ulcerations of the skin and mucous
• Photophobia membrane.
• Conjunctivitis
• Corneal ulceration • Initially the disease produces rapidly developing
• Uveitis round, erythematous macules, papules or
• Blindness due to secondary infection. vesicles over the skin.

Genital Lesions • These classic dermal lesions of erythema multi-
forme often characteristically appear as “target”,
• Urethritis “iris” or “bull’s eye”, etc.
• Balanitis
• Vaginal ulceration. • Vesicles also appear over the oral mucosa, which
rupture and produce diffuse, are extremely
HISTOPATHOLOGY painful irregular ulcers with bleeding tendency.

• The microscopic findings of erythema multi- • Stevens-Johnson Syndrome is the most severe
forme are often nonspecific, and they usually form of Erythema multiforme; which simulta-
consist of acanthosis, intra- or intercellular neously affect skin, mucosa, eyes and genitalia.
edema alongwith widespread necrosis of the
basal keratinocytes, etc. • The disease can cause trachea-bronchial
ulceration and pneumonia.
• Vesicles or bullae may form within the
epithelium or at the epithelium-connective • Sometimes erythema multiforme indicates the
tissue junction. possibility of internal carcinoma.

• Subepithelial connective tissue shows edema TREATMENT
and perivascular infiltration of lymphocytes,
neutrophils, eosinophils, macrophages, etc. Topical and systemic steroid therapy, coupled
with antibiotics.
DIFFERENTIAL DIAGNOSIS
DERMATITIS HERPETIFORMIS
• Acute primary herpetic stomatitis
• Aphthous ulcers Dermatitis herpetiformis is a rare chronic
• Pemphigus recurrent mucocutaneous disease of unknown
• Pemphigoid etiology. The disease has some connection with
• Erosive lichen planus. the dietary gluten.

CLINICAL FEATURES

• The disease commonly occurs among middle-
aged males.

• Initially there is pruritis with severe burning
sensation in the affected areas of skin.

• Gradually, there is appearance of erythe-
matous, papules or vesicles on the skin or
mucous membrane.

• These lesions are commonly seen over the
extremities, buttock, face, scalp, etc. and they
often resemble Herpes simplex.

• The vesicular lesions often develop in crops
and are symmetrically distributed.

• Oral lesions appear initially as vesicles but
soon they rupture and leave painful ulcers.

Oral Aspects of Dermatological Disorders 549

HISTOPATHOLOGY cells named “corps grains” and esoinophilic
cells named “corps ronds”.
• Disruption of the superficial epithelium with • Sometimes intra-epithelial bullae may appear
subsequent ulcerations. with acantholysis and suprabasilar split and
in such cases the lesions resemble pemphigus
• Aggregation of eosinophils and neutrophils vulgaris.
at the apex of the connective tissue papilla • The epithelial rete ridges are narrow, elonga-
with formation of microabscesses. ted and they often have a ‘taste-tube’ like
shape.
• Subepithelial bullae or vesicle formation with • The epithelium is often hyperplastic with
destruction of basement membrane. presence of chronic inflammatory cell
infiltration in the lamina propria .
TREATMENT
TREATMENT
Gluten-free diet and administration of Sulfapy-
ridine. By high doses of vitamin A therapy or steroids.

KERATOSIS FOLLICULARIS ACANTHOSIS NIGRICANS

Keratosis follicularis is a hereditary disorder of Acanthosis nigricans is a rare cutaneous disease,
the skin and is characterized by the formation of which usually affects the flexural surfaces of
multiple crusted, greasy lesions that often skin and it has an occasional oral mucosal
produce foul odor. component.

CLINICAL FEATURES TYPES

• The lesions commonly develop over the face The disease usually has three types:
and neck of the young individuals.
Benign type: Present at birth or during puberty.
• In the oral cavity, small white papules may It is inherited as autosomal dominant trait and
appear on the gingiva, tongue, hard palate, is never associated with internal malignancy. The
cheek, etc. benign type of acanthosis nigricans often occurs
in association with various disease conditions,
• The erythematous hyperkeratotic papules e.g. diabetes mellitus, hypothyroidism, acro-
develop on the skin over the trunk and scalp. megaly, Crouzon syndrome, chronic steroid
These usually measure about 2 to 3 mm in therapy, etc.
diameter.
Malignant type: Usually, develops after the age
• The papules become grayish-brown with age. of 40 years and is invariably associated with
• Smaller lesions coalesce together and thus internal malignancies like adenocarcinoma or
lymphoma of the GI tract.
gradually enlarge with time.
• Accumulation of keratin often gives a rough Pseudoacanthosis type: It is the most common
form of acanthosis nigricans and in this type,
texture to the lesion. Moreover, bacterial lesions develop around body creases as a result
desquamation of these keratin often produces of obesity.
foul smell in the lesion.
• Nails often have longitudinal lines or painful CLINICAL FEATURES
cracks.
• Skin lesions clinically present numerous, fine,
HISTOPATHOLOGY velvety, confluent plaques with brownish
pigmentation.
• Lack of cohesiveness among the surface
epithelial cells is the hallmark of keratosis • These lesions predominantly occur on the
follicularis; and the disease microscopically flexural surfaces of skin over axilla, palms,
reveals the presence of multiple clefts and soles, face, etc.
lacunae within the epithelium.

• Often these clefts or lacunae contain two types
of cells. The grain-like keratinized epithelial

550 Essentials of Oral Pathology

• Oral lesions are mostly seen in association ORAL MANIFESTATIONS
with the malignant form of the disease. • Vesicles and bullae develop over the oral

• Dorsum of the tongue exhibits hypertrophy mucosa especially in the tongue and cheek;
of the filiform papilla with development of a these lesions are followed by mucosal erosions
shaggy appearance. and subsequent development of white
leukoplakic patches.
• Tongue also presents some areas of papillo- • Oral mucosa may also have red, macular
matous growth. lesions or erythroplakic patches with super-
added candidal infections.
• Lip is grossly enlarged (mainly the upper lip) • The leukoplakia lesions in the oral mucosa
and its surface is dotted with small papillo- may turn into squamous cell carcinoma.
matous nodules especially at the commissure. • Severe periodontal tissue destruction and loss
of alveolar bone is often seen.
• Gingiva also exhibits hyperplastic changes
and the lesions are clinically similar to that of HISTOPATHOLOGY
the fibromatosis gingivae. • Oral mucosa exhibits hyperortho or hyper-

• Buccal mucosa and palate exhibit a velvety parakeratosis and acanthosis.
white appearance with papillary projections • In many cases there is presence of epithelial
in some areas.
dysplasia.
• The pigmentations which are seen in the • Skin lesions show increased vasculitis and
cutaneous lesions of the disease are often
absent in the mucosal areas. increased number of melanin containing
chromatophores.
HISTOPATHOLOGY
DIAGNOSIS
• Thickening of the epithelium with marked Presence of anemia, leukopenia, thrombocyto-
acanthosis. penia, Fanconi’s syndrome, etc.

• Hyperorthokeratinization of the surface TREATMENT
epithelium. No treatment is needed. Periodic evaluations of
the oral lesions are required to look for any
• Malignant form of the disease shows marked suspected malignant transformation.
epithelial hyperplasia and acanthosis.
WHITE SPONGE NEVUS
TREATMENT
White sponge nevus is a hereditary skin disease
There is no definitive treatment for this condition. characterized by the occurrence of white,
thickened, corrugated mucosal lesions in the oral
DYSKERATOSIS CONGENITA cavity that often affects several members of the
same family. The condition is genetically
It is a rare hereditary disorder of skin, which is determined and occurs due to a defect in the
inherited as a recessive trait. normal keratinization process of the oral
epithelium.
CLINICAL FEATURES
CLINICAL FEATURES
• The disease commonly occurs during early • The disease generally occurs at birth or during
childhood and it shows a definite male
predominance. childhood and there is no sex predilection.
• Clinically oral lesions exhibit symmetrically,
• Nails exhibit abnormal dystrophic changes
and they gradually shed in few days time. thickened, white, folded or corrugated
diffuse plaques, which develop bilaterally
• Skin shows grayish-brown pigmentations over the cheek.
over the trunk, neck and thighs.

• Facial skin appears red due to atrophic or
telangiectatic changes.

• Patients may also have mental retardation,
thrombocytopenic purpura, aplastic anemia,
dysphagia, deafness and hyperhydrosis of the
palms, soles, etc.

Oral Aspects of Dermatological Disorders 551

• The other common intraoral sites of this lesion Fig. 19.5A: White sponge nevus
include the palate, labial mucosa, tongue,
gingiva, floor of the mouth, etc. epithelium with acanthosis and intercellular
edema.
• Sometimes, these lesions may even develop • There may be presence of some large “vacuo-
in relation to the nasal, rectal or genital lated” cells in the spinus cell layer having
mucosa, etc. pyknotic nuclei.
• An eosinophilic condensation is sometimes
• The oral lesions are asymptomatic, soft and noticed in the perinuclear region of the
spongy and they have a peculiar opalescent superficial epithelial cells.
hue. • The abnormally prominent epithelial cell
membranes produce a typical “basket-
• Sometimes the shaggy white thickening can weave” appearance.
involve the entire oral mucosa. • The underlying connective tissue shows mild
or no inflammatory cell infiltrations.
• The surface of the lesion sometimes exhibit • Parallel striae of condensed parakeratin
areas of desquamation. traverse the surface layers in oblique planes.
• There is no epithelial atypia or dysplasia seen
• The border of the lesion is not clearly demar- in the epithelium.
cated and the edges fade away gradually in • Individual cell keratinization may be seen in
the normal oral mucosa. the spinus layer.

Key points of white sponge nevus TREATMENT

• White sponge nevus is a hereditary skin disease; No treatment is required for this self-regressing
which affects several members of the same disease.
family.
POLYMYOSITIS
• Oral lesions produce symmetric, thickened,
white, folded or corrugated diffuse plaques; Polymyositis is an inflammatory myopathy of
which occur bilaterally over the cheek. unknown etiology, which commonly affects the
skin.
• The oral lesions have a peculiar opalescent hue
and microscopically the disease presents mild to CLINICAL FEATURES
moderate hyperparakeratosis of the epithelium.
• The acute form of the disease affects the people
• Thickening of the spinus cell layer and inter- between the ages of 50 to 60 years while the
cellular edema often seen and there may be chronic form of the disease occurs in children
presence of some large “vacuolated” cells in the between the age of 5 to 15 years.
spinus layer.
• In both forms, there is a definite female
• The abnormally prominent epithelial cell predilection observed.
membranes produce a typical ‘basket-weave’
appearance.

• This is a self regressing disease and no treatment
is required.

DIFFERENTIAL DIAGNOSIS

• Hereditary benign intraepithelial dyskeratosis
• Lichen planus
• Leukodema
• Hyperplastic candidiasis
• Leukoplakia
• Verrucous carcinoma

HISTOPATHOLOGY (FIG. 19.5A)
• White sponge nevus microscopically presents

mild to moderate hyperparakeratosis of the

552 Essentials of Oral Pathology

• The disease clinically presents muscle pain, reaction, that eventually causes destruction and
fever, malaise, arthralgic pain , weight loss, dissolution of the desmosomal attachments
etc. between the cells leading to the loss of adhesion
between one cell to the other (acantholysis).
• A violaceous rash commonly develops over Further accumulation of fluid in the region
the cheek, eyelids and hands, etc. eventually leads to the development of intra-
epithelial bullae and a suprabasilar split in the
• Death often occurs due to cardiorespiratory epithelium.
failure or infections.
According to another theory, which is known
• Oral mucosa reveals erythematous patches as the “protease theory”, deposition of auto-
having lichen planus like appearance with antibodies within the epithelium induces a
swelling. proteolytic activity by activating the tissue
plasminogens. This in turn generates proteolytic
• Sometimes mucosal lesions may transform enzyme called “plasmin” that destroys the
into malignancy and moreover some lesions desmosomes.
of polymyositis may be associated with an
internal carcinoma. Note
• Autoantibodies: When specific antibodies are
DIAGNOSIS
produced against some of the body’s own
• Hypergammaglobulinemia tissues or cells or cellular components.
• Positive Rheumatoid factor • In case of pemphigus the antibodies are
• Positive antinuclear factor. produced against the antigenic components
of the desmosomes or the intercellular cement
TREATMENT substances (desmoglein-3 and desmoglein-1).
• These autoantibodies attach to the desmo-
There is no specific treatment for this condition. somal components and inhibit the molecular
reaction that is responsible for cell to cell
PEMPHIGUS binding in the epithelium.

Pemphigus is a group of vesiculobullous lesions CLINICAL FEATURES OF PEMPHIGUS
of the skin and mucosa membrane, which is (FIGS 19.6 TO 19.10)
characterized by the formation of intraepithelial
vesicles or bullae causing separation of the Pemphigus Vulgaris
epithelium above the basal cell layer.
• It is the most common type of pemphigus,
Types of pemphigus which occurs between the ages of 40 to 70
years and it is more prevalent among females.
• Pemphigus vulgaris
• Pemphigus vegetans
• Pemphigus foliaceus
• Pemphigus erythematosus
• Brazilian pemphigus

ETIOPATHOGENESIS Fig. 19.6: Pemphigus-I

An “autoimmune mechanism” plays the major
role in the development of pemphigus. Actually
the patient develops immunoglobulin G (IgG)
and complements in his or her body, which are
specifically targeted against the intercellular
cement substances (desmosomes) of the skin and
mucous membrane.

Deposition of such autoantibodies in the skin
or mucous membrane initiates an immune

Oral Aspects of Dermatological Disorders 553

Fig. 19.7: Pemphigus vulgaris of tongue Fig. 19.10: Pemphigus vulgaris of the oral mucosa
Fig. 19.8: Pemphigus-II
• Oral lesions often begin as “bleb-like” blisters
Fig. 19.9: Pemphigus vulgaris of lip or as diffuse gelatinous plaques.

• Soon rapidly developing vesicles or bullae on
several areas of the skin and mucous mem-
brane are found.

• The vesicles contain clear fluids initially but
later on there is formation of pus.

• Often these lesions develop in the mouth and
then spread widely on to the skin.

• The vesicles or bullae are fragile and rupture
almost as soon as they form.

• Ruptured vesicles in the oral cavity often leave
extremely painful, superficial, erythematous
ulcers with ragged borders.

• The ulcers often bleed profusely and may be
covered with blood-tinged exudates.

• Gentle traction or oblique pressure on the
unaffected areas around the lesion causes
denudation or stripping of the normal skin or
mucous membrane, this phenomenon is
known as “Nikolsky” sign”.

• Skin lesions in pemphigus vulgaris usually
appear over the scalp, trunk and umbilical
area.

• Oral lesions of pemphigus vulgaris can be
fulminating in nature since they not only
exhibit widespread involvement of the oral
mucosa but they can also spread rapidly to
eyes and skin.

• Sometimes, the disease involves the entire
body and in these cases the patient’s condition
becomes as serious as a severely burnt case,
especially in terms of fluid loss and risk for
secondary infections.

554 Essentials of Oral Pathology

• Patient may die of dehydration and septice- Fig. 19.11: Photomicrograph of pemphigus vulgaris
mia, etc. showing suprabasilar split of the epithelium

• Skin lesions of pemphigus usually heal by scar Brazilian Pemphigus
formation but the mucosal lesions heal
without scarring. However, oral mucosa often It is an endemic form of pemphigus, which
shows the formation of keratotic lesions affects several members of the same family and
following healing. is commonly seen in the costal areas of Brazil.

• Oral lesions in pemphigus vulgaris may HISTOPATHOLOGY OF PEMPHIGUS
involve any part of the oral cavity but are more
frequently found in those areas which are Pemphigus is, histopathologically, characterized
often subjected to trauma, e.g. cheek, labial by the following features:
mucosa, ventral surface of tongue, palate • Formation of the vesicle or bullae within the
gingiva, etc.
epithelium that often results in a supra-basilar
• Some ulcers can be very large in size causing spilt or separation (Fig. 19.11).
severe pain and they usually take much longer • Following this suprabasilar spilt in the epi-
time to heal. thelium, the basal cell layer remains attached
to the lamina propria, and it often appears as
• In over 60 percent cases of pemphigus a row-of-tomb stones.
vulgaris, oral lesions precede the skin lesions • Loss of intercellular bridges and collection of
and in about 25 percent cases, they are the only edema fluid result in acantolysis within the
manifestations of the disease. spinus cell layer, which causes disruption of
the prickle cells.
• Patients with oral pemphigus vulgaris often • As a result of acantholysis, clumps of large
have sore mouth with severe pain, excessive hyperchromatic epithelial cells desquamate
salivation, bleeding, difficulty in taking food that are often seen lying free within the
and an extremely foul smell in the mouth. vesicular fluid, these desquamated cells are
often rounded and smooth in appearance and
• In some cases of pemphigus vulgaris, ocular are known as “Tzanck cells”.
lesions may develop in the form of bilateral • Small number of polymorphonuclear neutro-
conjunctivitis. phil (PMN) and lymphocytes may be found
within the vesicular fluid, but there is
Pemphigus Vegetans

This is a common form of pemphigus lesion,
which resembles the pemphigus vulgaris in all
respects, but it has been observed that a
papillomatous hyperplasia (vegetation) often
occurs in this lesion following the rupture of
the bullae.

Oral lesions are present in about 50 percent of
cases of pemphigus vegatans.

The so-called ‘cerebriform tongue’ is a typical
clinical sign of this disease.

Pemphigus Foliaceus and Pemphigus
Erythematosus

Both are rare forms of pemphigus lesions and
here the bullae formation is often associated with
marked erythema of the involved skin. Both
these lesions resemble exfoliative dermatitis and,
rarely, there are any oral manifestations.

Oral Aspects of Dermatological Disorders 555

minimum inflammatory cell infiltration in the DIFFERENTIAL DIAGNOSIS
underlying connective tissue (unlike any other
vesiculobullous lesion). • Pemphigoid
• Erythema multiforme
IMMUNOHISTOCHEMISTRY • Bullous lichen planus
• Dermatitis herpetiformis
• In pemphigus, immunofluoresence tests of • Desquamative gingivitis
the perilesional tissue often demonstrate • Toxic epidermal necrolysis
the deposition of specific immunoglobulines • Aphthous ulcers
(IgG), within the intercellular areas of • Epidermolysis bullosa.
epithelium.
TREATMENT
• The intensity of the fluorescence is the greatest
in the suprabasilar region (spinous cell layers), • High dose of steroids
and gradually it becomes decreased towards • Immunosuppressive drugs, e.g. azathioprine
the surface of the epithelium.
or methotrexate
• Circulating autoantibodies against epithelial • Antibiotics to prevent secondary infections
intercellular desmosomes are of disgnostic • Fluid and electrolyte balance must be strictly
importance.
maintained.
Key points of pemphigus
PEMPHIGOID
• Pemphigus is a vesiculobullous lesion of the skin
and mucosa membrane; which occurs due to Pemphigoid is a group of relatively uncommon,
autoimmune destruction of the intercellular autoimmune vesiculo-bullous lesion; characte-
cement substance between cells. rized histologically by the subepithelial bullae
formation in the basement membrane zone
• The disease produces rapidly developing vesicles (BMZ) of the skin and epithelium.
or bullae on several areas of the skin and mucous
membrane. PATHOGENESIS

• Ruptured vesicles in the oral cavity often leave Progression of the disease pemphigoid occurs in
extremely painful, superficial erythematous the following mechanisms:
ulcers with ragged borders.
Formation of autoantibodies in the body against
• Gentle traction or oblique pressure on the the antigenic components of the basement
unaffected areas around the lesion causes membrane zone.
stripping of the normal skin or mucous
membrane, this phenomenon is known as ↓
“Nikolsky’s sign”. Binding of autoantibodies to hemidesmosome

• Histologically, the disease is characterized by associated antigens in the basement membrane.
formation of intraepithelial vesicles or bullae, ↓
which causes separation of the epithelium above
the basal cell layer. Activation of complements, which attract
inflammatory cells.
• Loss of intercellular bridges and collection of ↓
edema fluid result in acantholysis within the
spinus cell layer. Synthesis of chemotactic factors.

• Disruption of the spinus cells causes clumps of ↓
large hyperchromatic epithelial cells accumula-
ting within the vesicular fluid; these desqua- Release of proteolytic enzymes (especially
mated cells are known as “Tzanck cells”. protease) by inflammatory cells.
↓
• The disease is treated by high dose of steroid
therapy. Protease causes destruction of hemidesmosomal
junctions of the basement membrane zone.

↓

Formation of subepithelial vesicles or bullae.

556 Essentials of Oral Pathology

Unlike pemphigus, pemphigoid lesions Fig. 19.12: Ulcerative lesions of gingiva
usually heal up by scar formation (cicatri- in a patient with pemphigoid
cation). However, the scarring mostly occurs in
eye lesions and sometimes it can cause even • Bleeding into the bullae can make them look
blindness. like blood blisters.

Although a large number of clinical variants • Once the bullae rupture, they leave large
of pemphigoid exist, the most common and the painful, eroded or ulcerated areas, which heal
best understood among them are: (A) cicatricial up slowly (Fig. 19.12).
pemphigoid, and (B) bullous pemphigoid.
• Further erosions may occur in the adjoining
The former lesion occurs commonly in relation areas and the process continues for long.
to the mucous membrane, while the later one
occurs frequently in relation to the skin. • The gingival lesions are very common (seen
Pemphigoid is seen twice as common in females in about 90 percent cases) and they charac-
as in males and the mean age of occurrence is teristically present either patchy erythema
above 60 years. The disease is not life-threatening with mild discomfort or severe erythema,
and though, it often runs a chronic course for widespread desquamation, ulceration, etc.
many years.
• Sometimes these gingival lesions are the only
Cicatricial Pemphigoid manifestations of pemphigoid and the so
called desquamative gingivitis can be the
Cicatricial pemphigoid is also known as benign only manifestation of the disease.
mucous membrane pemphigoid (BMMP), and it
frequently affects the middle-aged or elderly • Pain and bleeding are the common complaints
females. and Nikolsky’s sigh is typically positive.

The most common site of occurrence is the oral • Irritation from denture, calculus and plaque
mucous membrane, followed by the conjunctiva. can exacerbate the condition.
Sometimes the disease also involves the nose,
pharynx, larynx, esophagus, genitalia, etc. • Secondary infection often occurs in pre-
existing pemphigoid lesions and oral lesions
Clinical Features usually heal up by scar formation.

Oral Lesions • Involvement of the pharynx, esophagus and
larynx may produce discomforts like sore
• In the oral cavity, cicatricial pemphigoid throat, dysphagia, strictures, etc. some
usually produces slowly progressive, mild patients may develop septicemia.
erosion or desquamation of the gingival
tissue. Eye Lesions

• Vesicles or bullae arise from mucosal areas, • Conjunctivitis.
which become erythematous earlier (Fig. 19.13). • Blister formation in the eye.
• Corneal ulceration.
• In severe cases, large vesicles or bullae may • Swelling of the fornix and corneal opacity.
develop on the palate, cheek, alveolar mucosa
or tongue, etc.

• Bullae are sometimes quite large in size and
they persist for several days (In contrast, the
vesicles or bullae in pemphigus do not persist
and they rupture as soon as they form).

• Skin lesions occur in about 5% cases and muco-
sal lesions always precede the skin lesions.

• The mucosal bullae are often tense and are
relatively tough because these are usually
covered by a full thickness epithelium.

Oral Aspects of Dermatological Disorders 557

Fig. 19.13: Vesicle formation near the Fig. 19.14: Vesiculobullous lesion of
inner canthus of eye in pemphigoid skin in pemphigoid

• Fibrosis and scarring of the lacrimal ducts, • Bullous pemphigoid having oral lesions may
which often leads to “dry eye”. be associated with internal malignancy.

• Scarring may cause adhesions between the Histopathology of Pemphigoid
bulbar and the palpebral conjunctiva. • Extracellular edema and vacuolation in the

• Scarring of the conjunctival mucosa results in basement membrane zone are the earliest
blindness. histological changes in cicatricial pemphi-
goid.
Skin Lesions • Gradually, there is formation of subepithelial
vesicles or bullae (Fig. 19.15).
The skin lesions are rare in cicatricial pemphigoid • The subepithelial bullae cause separation of
and occasionally few large, tense and fluid-filled the full thickness epithelium from the under-
vesicles or bullae may appear over the face, scalp lying lamina propria and thus the epithelium
or neck. forms the roof of an intact bullae till it is
ruptured.
Bullous Pemphigoid • Acantholysis and epithelial degenerative
changes are absent.
Bullous pemphigoid occurs more commonly on • Polymorphonuclear neutrophils (PMN) may
the skin and it seldom affects the oral mucosa. be found within the vesicular fluid.

Clinical Features Fig. 19.15: Photomicrograph of pemphigoid showing sub-
epithelial bullae formation
• These lesions also follow the usual clinical
patterns of vesiculation, followed by ulcera-
tion and finally healing (without scarring).

• Elderly people in the age group 70 to 80 years
are usually affected, and there is no gender
predilection.

• The skin lesions mostly occur over the trunk
and limbs, and usually undergo spontaneous
regression (Fig. 19.14).

• Skin lesions begin as red, eczematous plaques
which eventually progress to the formation of
bullae.

• Skin lesions always precede the mucosal
lesions and these mucosal lesions are always
smaller than that of the cicatricial pemphigoid.

558 Essentials of Oral Pathology

Key points of pemphigoid A. Hereditary epidermolysis bullosa
B. Acquired epidermolysis bullosa.
• Pemphigoid is a relatively uncommon, auto- The hereditary forms of epidermolysis bullosa
immune vesiculobullous lesion. It is characterized include epidermolysis bullosa simplex, epider-
by subepithelial bullae formation in the molysis bullosa dystrophic and junctional
basement membrane zone (BMZ). epidermolysis bullosa.
There is only a single form of acquired epider-
• The disease produces large vesicles or bullae in molysis bullosa, and it is named as epidermolysis
the skin and mucous membrane; which rupture bullosa aquisita. This acquired from of the
after a certain period and leave painful ulcers. disease may be associated with multiple
myeloma, diabetes mellitus, tuberculosis,
• Pain, bleeding and secondary infection are the amyloidosis, crohn’s disease, etc.
common problems caused by these ulcers.
CLINICAL FEATURES
• Nikolsky’s sigh is typically positive in this disease.
• Besides the dermal lesions eye lesions also • All forms of the disease are characterized by
the formation of multiple vesicles or bullae on
commonly develop in Pemphigoid. the pressure areas of the skin (i.e. elbows and
• The eye lesions include conjunctivitis, blister for- knees).

mation in the eye, corneal ulceration, fibrosis of • Dystrophic type is the most severe from of the
the lacrimal duct leading to dry eye, etc. disease and it may cause death secondary to
• Histologically, pemphigoid presents formation of septicemia.
subepithelial vesicles or bullae at the basement
membrane zone which causes separation of full • The bullae rupture and leave raw, painful
thickness epithelium from the underlying ulcers, which heal up with scarring.
connective tissue.
• Systemic steroid therapy is the treatment of • The healing of skin lesions cause extensive
choice. scarring with pigmentation or depigmentation
of the area.
• Subepithelial connective tussue shows
inflammatory cell infiltration by lymphocytes, • Nails often shed or exfoliate due to formation
macrophages and eosinophils in the perivas- of blisters in the nail beds.
cular areas.
• The hereditary forms of the disease are usually
• Blood vessels are often dilated and prominent very severe in nature and occur during infancy
in the superficial part of the lamina propria. or early childhood, whereas the acquired form
of the disease is common during adulthood
Treatment only.

Systemic steroid therapy is the treatment of • Patients with hereditary epidermolysis bullosa
choice in both forms of the disease. may also exhibit stunted growth, mental
retardation, ectodermal dysplasia, etc.
EPIDERMOLYSIS BULLOSA
• The patients may have alopecia and claw-like
Epidermolysis bullosa is a generalized desqua- hands due to repeated scarring and contractures.
mating condition of the skin and mucosa which
is often associated with scarring, contractures • Many such patients die during childhood.
and dental defects.
ORAL MANIFESTATION
In epidermolysis bullosa, there is a separation
of the epithelium from the underlying connective • Oral lesions are particularly common and
tissue with formation of large blisters, which severe in relation to the hereditary forms of
often heal with extensive and often immobilizing the disease.
scar formation.
• The features include rapidly developing,
Several forms of epidermolysis bullosa have multiple, fragile and hemorrhagic blisters or
been recognized, and these lesions are divided bullae in the areas of trauma (particularly in
into two broad categories: the palate).

Oral Aspects of Dermatological Disorders 559

• The lesions rupture soon and leave painful PATHOGENESIS
ulcers, which later on heal by scarring.
Pathogenesis of lupus erythematosus is not
• Repeated blistering and scarring around the clearly known. It is believed that autoimmune
oral cavity result in decreased mouth opening reactions cause changes within the basal cells of
(microstomia), ankyloglossia and loss of the skin or mucous membrane along with the
vestibular sulci. collagen and vascular tissues. Autoantibodies to
DNA and other nuclear and ribonuclear protein
• Some patients may develop perioral and antigens are present in blood. The circulating
perinasal crusted and hemorrhagic granular autoantibodies cause cross reactivity with
lesions. antigenic determinants on multiple tissues.

• Sometimes oral lesions may transform into The disease occurs in two basic forms.
squamous cell carcinoma. A. Systemic lupus erythematosus (SLE), and
B. Discoid lupus erythematosus (DLE).
• Hypoplastic pitted enamel of the molar teeth
may occur in a few cases of epidermolysis Systemic Lupus Erythematosus (SLE)
bullosa.
SLE frequently produces lesions in the skin and
• Patients may exhibit delayed eruption of oral mucous membrane, and besides this, it also
tooth, increased caries susceptibility and involves certain other body systems.
increased periodontal diseases.
Clinical Features of SLE
HISTOPATHOLOGY
Skin lesions
• Extensive destruction of the basal or the
suprabasal layers of the oral epithelium, • Systemic lupus erythematosus (SLE) occurs in
results in the formation of vesicles or bullae. about 0.1 to 0.4 percent individuals.

• Bullae formation may also be seen within • Skin lesions of SLE are characterized by the
enamel organ of the developing tooth germ. development of fixed, erythematous rashes
that often have a “butterfly configuration”
• Mucosal bullae formation occurs at different (Fig. 19.16) over the malar region and across
planes in various forms of epidermolysis the bridge of the nose.
bullosa and these are explained in the
following sections. • In some cases, the rashes may spread
diffusely and have a wide area of skin
Mucosal bullae formation involvement.
Disease types Level at which bullae forms
• These skin rashes produce itching or burning
Simplex type Intraepithelial bullae. sensation, which is often aggravated by the
Junctional type At the level of lamina lucida
Dystrophic type At the level of sublamina densa
Acquired type At the level of sublamina densa.

TREATMENT

• Systemic steroid therapy
• Immunosuppressive drug therapy
• Avoidance of trauma.

LUPUS ERYTHEMATOSUS Fig. 19.16: SLE patient showing erythematous
rash on the facial skin
Lupus erythematosus is an autoimmune dis-
order, characterized by the destruction of tissue
due to the deposition of autoantibody and
immune complexes within it. Production of
autoantibody, particularly the antinuclear
antibody is the hallmark of lupus erythematosus.

560 Essentials of Oral Pathology

Fig. 19.17: SLE patient showing loss of hair over the scalp Fig. 19.18: Intense erythema in the oral mucosa with
areas of ulcerations in SLE patient
exposure to sunlight. The disease often causes
hyperpigmentation of the skin. Oral Lesions
• The diseases affect females five times more
often than males and it is mostly detected in • Oral lesions occur in about 20 percent cases of
the fourth decade of life. SLE and these lesions are usually white,
• Patchy or extensive loss of hair from the scalp hyperkeratotic plaque-like areas and resemble
is a very common (Fig. 19.17) clinical finding lichen planus.
in lupus erythematosus (hair may grow once
again after successful treatment in case of SLE, • Oral and nasopharyngeal ulcerations are reco-
but not in case of DLE). gnized as the major diagnostic manifestations
of SLE (Fig. 19.18).

• Occasionally, there may be presence of ery-
thematous lesions in the oral cavity.

General problems Systemic manifestations of SLE
Fever, weight loss, fatigue, malaise, vomiting, diarrhea, anorexia,
Skin lymphadenopathy, etc.

Serous membrane Rashes with itching sensation and the rash often have butterfly
Lung problem configuration
G I Tract involvement
Ocular disturbances Pleurisy and endocarditis
CNS disturbances
Hematologic disorders Pneumonitis
Cardiac problems
Pancreatitis, hepatomegaly and splenomegaly
Renal problems Conjunctivitis and retinal damage

Joint problems Neuroses, psychoses, depression, strokes and cranial nerve palsies.
Mouth
Disease often coexisting Anemia, purpura and leukopenia
with SLE
Vegetations in the heart valves (Libman-Sacks endocarditis) and
myocarditis.
Profuse proteinurea, nephritic syndrome and fibrinoid degeneration
of the glomerular capillaries, which may lead to renal failure.

Non-deforming arthritis and arthralgia

Stomatitis and Sjogren’s syndrome

Sjogren’s syndrome, Raynaud’s phenomenon, scleroderma, pemphigoid,
erythema multiforme and pemphigus

Oral Aspects of Dermatological Disorders 561

• Often there is formation of hemorrhagic • DLE clinically presents multiple, white plaques
macules in the oral mucosa that becomes with central atrophy, shallow ulcers, reddish
frequently ulcerated. purple erosions, etc.

• Ulcerated lesions are often painful and are • Histologically, SLE produces atrophy with
surrounded by a red halo. hyperkeratinization and liquefactive degene-
ration of the basal cell layer of epithelium.
• Severe burning sensation in the oral mucosa
is often present and the affected area is • Systemic steroid therapy is the treatment of
extremely tendered to palpation.
choice in both forms of the disease.
• Oral lesions mostly occur over the buccal
mucosa, palate, gingival, etc. Histopathology

• The vermillion border of the lower lip is some- The oral lesions present the following histopatho-
times affected and the condition is known as logical findings:
“lupus cheilitis”. • Atrophy with hyperkeratinization of the oral

• Purpuric lesions, e.g. petechiae and ecchymosis, epithelium
etc. are frequently seen in the oral mucosa. • Liquefactive degeneration of the basal cell

• Secondary infections frequently occur to layer
these mucosal lesions (mostly candidiasis). • Edema of the subepithelial connective tissue

• There may be involvement of salivary glands with vascular dilatations
in SLE, which causes secondary Sjogren’s • Lymphocytic infiltration and fibrinoid
syndrome and severe xerostomia.
degeneration of the collagen fibers.
• Besides xerostomia, patients may also suffer
from altered taste sensations, sore mouth, Discoid Lupus Erythematosus (DLE)
chronic periodontal disease, nasopharyngeal
ulcerations, etc. DLE is a dermal lesion in which, oral lesions are
also commonly seen and unlike SLE this disease
• In some cases, oral lesions may undergo is not associated with autoantibody productions.
malignant transformations. DLE also occurs commonly among females and
the usual age is the third and fourth decades of
Key points of lupus erythematosus life. The disease commonly involves the skin over
the back, chest and extremities, and the oral
• Lupus erythematosus is an autoimmune mucous membrane.

disorder, characterized by destruction of tissue Clinical Features of DLE

due to the deposition of autoantibody and Skin Lesions (Fig. 19.19)

immune complexes within it. • These are slightly elevated, red or purple
macules, which are often covered by a yellow
• The disease occurs in two forms- Systemic lupus or gray scale.

erythematosus (SLE) and Discoid lupus • Skin lesions of DLE also present a butterfly
distribution over the malar region and across
erythematosus (DLE). the nose.

• Systemic lupus erythematosus presents • Upon forceful removal of the covering scale,
numerous “carpet track” extensions of the
characteristic fixed, erythematous rashes with a pialo-sebaceous channels appear.

“butterfly configuration” over the malar region • Skin lesions in DLE usually enlarge at the
periphery and in some cases, epidermoid or
and across the bridge of the nose. basal cell carcinoma may develop from these
lesions.
• In the oral cavity SLE produces white,

hyperkeratotic plaque-like and resembles lichen

planus. Involvement of salivary glands in SLE

often results secondary Sjogren’s syndrome and

severe xerostomia.

• Systemic manifestations of SLE include

hepatosplenomegaly, pneumonia, cardiac

problem, renal problem, GI disturbances, neural

problem, etc.

562 Essentials of Oral Pathology

Fig. 19.19: Small erythematous lesions on the Fig. 19.20: Photomicrograph of mucosal
facial skin in a patient with DLE lesion of DLE

• Involvement of scalp with loss of hair is also • Hyper-ortho or parakeratinization of the
common. surface epithelium.

Oral Lesions • The epithelium may be atrophic in some cases.
• The oral lesions of DLE include multiple white • Few lesions exhibit keratin plugging, acantho-

plaques with central atrophy, shallow ulcers, sis and pseudo-epitheliomatous hyperplasia.
reddish purple erosions, etc. • Liquefactive degeneration of the basal cell
• These mucosal lesions frequently involve
cheek, lips, gingiva, palate, etc. layer.
• The lesions initiate at a particular point and • Basophilic degeneration of the collagen with
then gradually extend peripherally to cover a
wider area. sign of hyalinization and perivascular
• As the white plaque of the oral cavity extends lymphocytic infiltration in the lamina propria.
peripherally, its central area becomes red and • Inflammatory cell infiltration typically
ulcerated while the border remains white and extends deep into the connective tissue.
keratotic. • PAS positive thickening of the basement
• Small white dots are often present on the oral membrane zone and narrow blood vessels are
mucosa with a slightly elevated border. seen, which occur due to deposition of
• Pain and burning sensations are common in antigen-antibody complex.
the mouth in DLE. • Vasculitis occurs commonly in SLE but it is
• Small slit-like ulcers are often seen near the not seen in DLE.
gingival margin.
• Often these oral lesions resemble leukoplakia. LABORATORY INVESTIGATIONS OF
• Erythema or ulcerations in DLE may be LUPUS ERYTHEMATOSUS
surrounded by a white keratotic border, which Lupus erythematous (“LE”) cell inclusion
can also have radiating striae. phenomenon: It is a specific laboratory test for
SLE. If the serum from a patient suffering from
Histopathology (Fig. 19.20) SLE is added to the buffy coat of normal blood, a
Oral lesions of DLE present the following typical “LE” cell will develop. These are
histologic findings: neutrophil leukocytes, which have phagocytosed
other leukocytes. LE cell is characterized by a
large, circular, basophilic inclusion within a
neutrophil. Positive LE cell test is rare in DLE.

Oral Aspects of Dermatological Disorders 563

Other laboratory tests include the following: or multiple, red or ivory colored, smooth, hard
• Antinuclear antibodies are present in the patches on the skin. Although, facial skin is
frequently affected, it does not have any oral
serum. manifestations. Childhood “morphea” form of
• Anti-DNA antibodies are also present. scleroderma may be responsible for the
• Polyclonal hyperactivity of the B-lympho- development of facial hemihypertrophy.

cytes. The generalized or diffuse form of sclero-
• Decrease in the number of suppressor cells. derma usually occurs among children or young
• Blood examination reveals-leukopenia, adults, and it frequently affects females.
Scleroderma is more prevalent in areas where
thrombocytopenia and hemolytic anemia. silicosis is a common environmental hazard.
• Hypergamaglobulinemia and profuse protei-
Clinical Features of Diffuse Scleroderma
nuria. (Figs 19.21 and 19.22)

Direct immunofluorescence test: This test will • Females between the ages of 30 to 50 are very
reveal the deposition of immunoglobulins (e.g. frequently affected by scleroderma.
IgG, IgA, IgM and Complement 3) in the base-
ment membrane zone of the epithelium and skin, • The disease begins with edema of the skin over
in case of both SLE and DLE. the face, hands or trunk.

Indirect immunofluorescence test: Reveals pro- • The affected skin becomes thinned and stiff
minent circulating auto-antibodies. and it is fixed firmly to the underlying tissues,
e.g. muscles, bones, etc.
Treatment
• The affected skin is also marked by multiple
Systemic steroid therapy is the treatment of telangiectasias and contractures.
choice in lupus erythematosus.
• The skin often appears reddish and scaly and
SCLERODERMA it also frequently exhibits scarring.

Scleroderma or progressive systemic sclerosis is • Fixation and stiffness of the skin causes
a complex multisystem autoimmune disease progressive loss of mobility of hands and joints
characterized by progressive diffuse subcuta- as well as restricted movements of many
neous fibrosis (sclerosis) of the skin, widespread internal and external organs.
submucous fibrosis in oral cavity and disorders
in multiple internal organs including the G I tract,
lungs, heart , kidney, etc.

PATHOGENESIS

Blood circulation insufficiency in the tissue
because of the abnormalities in the arterioles and
blood capillaries; cause replacement of the
normal connective tissue by the dense collagen
bundles and this results in the fibrosis or sclerosis
of the tissue.

TYPES Fig. 19.21: Facial profile of a patient with scleroderma

Scleroderma usually occurs in two basic forms:
A. Localized or morphea form
B. Generalized or diffuse form (it is the more
common type).

The localized form of scleroderma is charac-
terized by fibrosis with development of single

564 Essentials of Oral Pathology

of fibrosis, and later on, the patient becomes
completely bed-ridden.
• Fibrosis of the viscera causes dyspnea,
dysphagia, pulmonary hypertension, loss of
vision, etc.
• Finally all the internal organs, e.g. GI tract,
heart, lungs, kidneys, etc. become affected by
the fibrosis and these organs gradually loose
their respective functions. Among which

cardiac failure is the most threatening.
• CREST syndrome is often associated with

scleroderma and it includes the following
component features:

Features of CREST syndrome

Fig. 19.22: Stiffness of the fingers in patient C—Calcinosis Cutaneous deposition of
with scleroderma cutis calcium salts

• Resorptions of terminal phalanges and flexion R—Raynaud’s When patient’s hands and feet
contractures often produce a typical “claw- phenomenon are exposed to cold tempera-
like” appearance of the fingers. In this ture, the digits become dead
situation, the patient is unable to straighten white in appearance initially
the fingers. due to vasospasm and later on
they become bluish due to
• Ulcerations may occur on the finger tips due venous stasis.
to fibrosis (abnormal collagen deposition) and
reduced vascularity. E—Esophageal Fibrosis of the esophageal
dysfunction submucosa with stricture
• Widespread involvement of the disease causes formation.
wasting of muscles and body fat, with
weakening of the limbs. S—Scleroductyly Abnormal collagen deposition
causes stiffness and flexure of
• Patients develop arthritis and arthralgia, along fingers with a claw-like defor-
with neuralgia and paresthesia of the skin. mity.

• As the disease progresses, the skin becomes T—Telangiectasia Bleeding from superficial
yellow, gray or ivory white in color and it dilated capillaries produces
shows a waxy appearance. multiple red macules on the
skin, especially on the face.
• The skin gradually becomes thin or atrophic
with areas of pigmentation and subcutaneous • Besides this, scleroderma may also coexist
calcium deposition (calconosis cutis). with some other systemic diseases like-
Sjogren’s syndrome, rheumatoid arthritis,
• Due to fixation or toughness of the facial skin, polymyositis and lupus erythematosus.
“wrinkles” (lines of facial expression) do not
form and eyes also become narrow. These Oral Manifestation of Diffuse Scleroderma
causes a “mask-like” appearance of the face.
This type of typical facial appearance or • Mask-like expressionless face (Mona Lisa
expression in scleroderma is also named as face) as wrinkles (lines of facial expression)
“Mona Lisa face” (Fig. 19.21). does not develop due to the stiffening of facial
skin.
• Atrophy of the ala of the nose often gives rise
to a “pinched” appearance of the nose; which • Multiple telangiectatic spots may be seen on
often produces a “mouse facies”. the facial skin.

• Restriction of movements occurs in all the
voluntary and involuntary muscles as a result

Oral Aspects of Dermatological Disorders 565

• Complete loss of facial esthetics and expres- Fig. 19.23: Stiffness of the oral mucosa with a blanched
sion. appearance in scleroderma

• Patient may have a “pinched” appearance of • Decreased salivary secretion causes dry
the nose, as the ala of the nose becomes mouth (xerostomia) and increased suscepti-
atrophic due to fibrosis this often gives the bility to dental caries and candidal
patient a typical “mouse facies”. infections.

• Intraorally, the disease mostly involves the • Gingival tissue becomes pale and firm. More-
tongue, cheek, lips, soft palate, larynx, etc. over there can be severe gingival recession due
to loss of attached gingiva. Alteration of the
• Fibrosis and stiffening of the periarticular fibrous components of gingiva may also result
in advanced periodontitis
tissue cause restricted movements of the TM
Joint with development of varying degrees of • Looseness of teeth as periodontal ligaments
“trismus” (Fig. 19.23). expands with more and more collagen
• Pain, clicking sounds and crepitations in the deposition and loss of alveolar bone.
temporomandibular joint.
• The restricted movements of the TM Joint may • Failure of implants is common in this disease
be due to fibrosis, muscle wasting, skin due to xerostomia and mucosal as well as
tightening, calcinosis, etc. submucosal tissue abnormality.
• The lips of the patient in scleroderma may
appear “pursed” (lips appear as if the patient • Weakness of hands, decreased mobility of the
is about to blow whistle by the mouth) with TM joint, decreased mouth opening,
radiating furrows and it occurs due to decreased salivary secretions, etc. lead to poor
oral hygiene.
constriction of the mouth aperture (micro-
stomia) resulting from fixation and rigidity of RADIOLOGICAL FEATURES
the lip muscles.
• Moreover the constricted lip of the patient • There is generalized widening or thickening
often produces a typical facial appearance; of the periodontal ligament space.
which is called “fish mouth”.
• Stiffening and restricted movements of the • Resorption of bone occurs in some areas of the
facial muscles and tongue cause difficulty in condyle or ramus of mandible due to persis-
speech. tent pressure from the contracting facial skin,
• Oral mucosa shows edema, which is often muscles and joints.
followed by atrophy and indurations.
• Atrophy of the minor salivary glands often • In untreated cases, severe mandibular bone
resorption occur, which can lead to patholo-
causes dry mouth (xerostomia). Moreover gical fracture and even development of
Sjogren’s syndrome may also be associated osteomyelitis.
with the disease in few cases.
• Stiffness of the tongue muscles cause narrow-
ing of the tongue (chicken tongue) with res-
tricted movements. This along with
xerostomia may cause difficulty in speech and
swallowing. The surface of the tongue often
looks smooth.
• Esophageal fibrosis or stricture causes
increased abnormal gastroesophageal reflux
which often results in dysphagia, heartburn

and severe erosions of tooth.
• Palatal rogue areas become flattened.
• Trigeminal neuralgia can also be associated

with scleroderma.

566 Essentials of Oral Pathology

Key points of oral manifestations of Scleroderma

Affected orofacial area Signs and symptoms

Lips “Pursed” lips with constriction of mouth opening

Mouth Microstomia

Face Mask-like expressionless face, no wrinkles on skin

Facial muscles Weakness and muscle wasting

Oral mucosa Edematous and atrophic, candidiasis often present

Salivary glands Decreased salivary secretion (xerostomia), Sjogren’s Syndrome

T M Joint Restricted movements with trismus, pain and clicking sound

Tongue Stiffness with difficulty in speech and swallowing

Esophagus Fibrosis and stricture causing increased gastro-esophageal reflex.

Gingiva Becomes pale and firm

Palate Loss or flattening of palatal rogue

Teeth Premature loss of teeth due to advanced periodontitis and alveolar bone loss

Nerves Trigeminal neuralgia (occasionally)

HISTOPATHOLOGY EHLERS-DANLOS SYNDROME

• Oral epithelium becomes atrophic with Ehlers-Danlos syndrome is a group of hereditary
flattening of the rete pegs. disorders characterized by defective or abnormal
collagen synthesis in various body organs.
• Areas of pigmentations are often present on
the epithelium. CLINICAL FEATURES

• Thickening and hyalinization of the collagen • The patient often exhibits hypermobility of
fibers occur in the connective tissue with joints and increased laxity or hyperelasticity
atrophy of the minor salivary glands. of the skin, and because of this, the patient is
often referred to as the “rubber man”.
• In the early stages, perivascular infiltrates of
mononuclear inflammatory cells are common. • Excessive bruising tendency and defective
wound healing occurs due to increased
• Blood vessels gradually become scanty and fragility of the skin and blood vessels
there is narrowing of the lumen of the remain-
ing vessels due to the perivascular fibrosis. • The skin is velvety, thin and hyperextensible.
Moreover, it shows an abnormal scarring
• The thickness of the periodontal ligament is response when subjected to minor injuries
increased due to the increased synthesis of (Papyraceous scarring); and the scarred area
collagen and oxytalan fibers. of skin appears as “crumpled cigarette
papers”.
• Sweat glands, sebaceous glands and hair
follicles are often absent in the skin. • Development of aortic aneurysm and its
rupture is the common life threatening factor
TREATMENT for the patient.

No specific treatment is available for sclero-
derma. Systemic steroid therapy may produce
partial remission.

Oral Aspects of Dermatological Disorders 567

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Different types of pain in the orofacial region

A. Pain in the teeth and supporting structures • Damage of TM Joint
• Pulpitis • Postoperative osteomyelitis
• Fracture of tooth • Damage to the nerve during treatment
• Cracked tooth syndrome
• Apical periodontitis E. Neurological pain
• Acute periapical abscess • Trigeminal neuralgia
• Pericoronitis • Herpes zoster
• Lateral periodontal abscess • Multiple sclerosis
• HIV-associated periodontitis • Post-herpetic neuralgia
• ANUG • Migrainous neuralgia
• Causalgia
B. Pain in the jaws • Intracranial tumors
• Fractures • Bell’s palsy
• Osteomyelitis • Glossopharyngeal neuralgia
• Infected cyst
• Osteoradionecrosis malignant neoplasms F. Pain from extraoral sources
• Sickle cell infarcts • Maxillary sinusitis
• Meningitis
C. Pain in edentulous patients • Carcinoma in maxillary antrum
• Trauma from denture • Acute parotitis
• Mucosal disease below the denture • Salivary calculi
• Jaw pathology • Sjogren’s syndrome
• Glossodynia • Malignant neoplasm of salivary gland
• Impacted tooth • Otitis media
• Broken root of tooth erupting under the • Cavernous sinus thrombosis
denture • Myocardial infarction
• Excessive vertical height of denture • Myofacial pain dysfunction syndrome (MPD)

D. Postoperative pain G. Psychological pain
• Dry socket • Atypical (psychogenic) facial pain
• Aerodontalgia • Burning mouth syndrome (MPD)
• Fracture of jaw

DISEASES OF THE NERVES The trigeminal nerve has three divisions- oph-
thalmic, maxillary and mandibular; out of these
TRIGEMINAL NEURALGIA maxillary and mandibular branches are almost
exclusively affected in trigeminal neuralgia.
Neuralgia can be defined as the pain along the
distribution of nerves and, therefore, trigeminal ETIOLOGY
neuralgia refers to the pain along the • Mostly idiopathic
distribution of any branch of the trigeminal • Traumatic compression of the nerve resulting
nerve. This is the most common neuralgia or
nerve pain disorder. in demyelination

570 Essentials of Oral Pathology

• Biochemical change in the nerve cells • However, right side of the face is affected
• Abnormal blood vessel causing compression more frequently than the left side and it rarely
crosses the midline.
of the nerve as it exists from the cranial fora-
men. • Patients live in fear of unpredictable painful
attacks, which result in insomnia, irritability,
CLINICAL FEATURES anticipatory anxiety and depression, etc.
Suicidal tendency is also not uncommon.
• This condition often causes severe, unilateral,
‘lancinating’ type of pain in the orofacial • Surprisingly there is no detectable disease or
region that is usually radiating in nature. pathology present in the jaw in patients with
trigeminal neuralgia.
• The disease usually occurs among middle–
aged people (above 35 years of age) and it HISTOPATHOLOGICAL FINDINGS
occurs more in females than males.
In trigeminal neuralgia, focal areas of myelin
• Most of the patients give the history of excru- degeneration can be seen within the gasserian
ciating pain attacks, which feels like sharp ganglion or along the course of the nerve.
stabbing or electric shock.
LABORATORY DIAGNOSIS
• The pain in trigeminal neuralgia lasts for only
a few seconds or minutes and then disappears • History of the patient and normal neurological
promptly. Between the attacks the people are findings usually establish the diagnosis
relatively of pain free. beyond doubt.

• Often the burst of pain attacks are precipitated • MRI may be suggested for detection of any
by touching some “trigger zones” on the face. space occupying lesion or any vessel compres-
Presence of trigger zone is a definitive sion in the nerve root.
indicator of trigeminal neuralgia.
• CT scan and MRI of the head and neck region
• Trigger zones may get stimulated and the pain are advised to rule out suspected brain tumor,
starts due to touching, washing the face, tooth meningitis or any other neurological abnor-
brushing, talking, eating, chewing, smiling, mality.
shaving or even if a strong breeze touches the
face. Key points of trigeminal neuralgia

• Thus, the people with trigeminal neuralgia • Trigeminal neuralgia refers to severe, excru-
become plagued by intermittent severe pain ciating, pain along the distribution of any branch
that interferes with common daily activities, of trigeminal nerve on one side of the face.
e. g. eating, sleep and shaving, etc.
• The ‘lancinating’ or excruciating pain in
• Patients try to avoid foods or drinks because trigeminal neuralgia lasts for only a few seconds
of the fear of horrific pain. or minutes and then promptly disappears.

• The pain in trigeminal neuralgia may produce • Often the burst of pain attacks are precipitated
spasmodic contractions of the facial muscles by touching some “trigger zone” on the face.
and because of this characteristic muscle Presence of this trigger zone is a definitive
spasms, the condition is often called ‘Tic indicator of trigeminal neuralgia.
doulorreux’.
• The trigger zones on the face may get stimulated
• The pain may involve any part of the face on by any of the common actions, e. g. washing the
either side, depending upon which branch of face, tooth brushing, talking, eating, chewing,
the trigeminal nerve is affected. Generally, smiling, shaving, etc.
maxillary branch is more often affected than
the mandibular branch (ophthalmic branch is • The disease occurs more frequently among
least affected). middle aged females and maxillary or
mandibular branch of the trigeminal nerve is
• If maxillary branch is involved the pain occurs often involved.
in the cheek bone, entire nose, upper lips and
teeth of upper jaw. Whereas, if mandibular • Pain in trigeminal neuralgia often characteris-
nerve is involved the pain occurs in lower tically produces spasmodic contractions of the
cheek, lower lip, lower teeth and jaw. facial muscles.

Diseases of the Nerves and Muscles 571

DIFFERENTIAL DIAGNOSIS • Sphenopalatine neuralgia occurs more fre-
quently among females and sometimes it may
• Multiple sclerosis be associated with other symptoms like
• Migraine sneezing, nasal discharges and watering of the
• Atypical neuralgia eyes, etc.
• Myofacial pain
• Cluster headache • Interestingly in some patients the onset of pain
• TM joint disorder is exactly at the same time every day and
• Intracranial hemorrhage hence, it is referred to as the “alarm clock”
• Acute pulpitis headache.
• Fractured tooth.
GLOSSODYNIA AND GLOSSOPYROSIS
TREATMENT
Glossodynia refers to the painful tongue, while
Trigeminal neuralgia can be treated by the glossopyrosis means burning sensation of the
following methods: tongue.
• Peripheral neurectomy.
• Injection of alcohol or boiling water into the Etiology of glossodynia and glossopyrosis

gasserian ganglion. • Vitamin deficiency
• Injection of steroid or anesthetic in the • Anemia (especially pernicious anemia)

ganglion • Hormonal disorder, e. g. diabetes, hypothyroi-
• Electrocoagulation of the same ganglion. dism
• Administration of carbamazepines and
• Xerostomia
phenytoin, etc. • GI disturbances (hyper or hypoacidity)
• A recent treatment called microsurgical • Psychogenic factor, e.g. cancer phobia, chronic

decompression of trigeminal root being tried anxiety and depression
with good results. • Trigeminal neuralgia
Unfortunately, none of these treatments can • Referred pain from tooth
produce impressive and permanent results. • Angioneurotic edema
• Heavy metal poisoning, e. g. mercurialism
SPHENOPALATINE NEURALGIA • Moeller’s glossitis
• Postmenopausal syndrome
Sphenopalatine neuralgia is a distinctive syn-
drome of headache; characterized by a unilateral • Oral thrush
radiating pain in the region of the eye, maxilla, • Cervical nerve injury
ears, teeth, cheek and nose, etc.
CLINICAL FEATURES
TYPES
• Glossodynia usually occurs among the
There are two types of sphenopalatine neuralgia middle-aged or elderly people, mostly
A. Episodic among females.
B. Chronic
• The sensations which are commonly encoun-
CLINICAL PRESENTATION tered in this disease are pain and burning,
itching or stinging sensations of the mucous
• The pain begins very rapidly; it persists for membrane covering the tongue.
few minutes to few hours and then
disappears. • However in glossodynia, the tongue appears
normal and clinically there are no apparent
• Generally 1 to 3 short attacks of pain occur in lesions, e.g. discoloration or ulcerations, which
a day. can precipitate the symptoms.

• The condition often simulates toothache, • The pain is low or absent during morning,
however, unlike the trigeminal neuralgia; no however, it gradually builds up as the day
“trigger zone” is present in this case. progresses.

572 Essentials of Oral Pathology

TREATMENT be the following—abnormal blood vessels
pressing down on glossopharyngeal nerve,
Topical anesthetics, analgesics, muscle relaxants growth at the base of the skull and tumor or
and sedatives, etc. are given in case of glosso- infection in the mouth, etc.
dynia, although permanent remission is difficult
to achieve. Low doses of benzodiazepines, BELL’S PALSY (FACIAL NERVE
tricyclic antidepressants or anticonvulsants may PARALYSIS)
also be effective.
Bell’s palsy refers to the paralysis of facial nerve
AURICULOTEMPORAL SYNDROME resulting in inability to control the facial muscles
(FREY’S SYNDROME) on the affected side of the face. The condition is
named after Scottish anatomist Charles Bell who
Frey’s syndrome occurs due to anomalous repair first described it.
of the damaged auriculo temporal nerve follow-
ing injury, which results in the innervations of Functions of facial nerve
sweat glands by the parasympathetic salivary
fibers. • Blinking
• Closing eyes
The patients with this syndrome typically • Smiling
exhibit flushing and sweating in the temporal • Frowning
area during meals. Moreover, this can happen • Lacrimation
even when the patient sees or thinks or talks • Salivation
about certain foods that cause increased sali- • Taste sensation from the anterior two-third of
vation. The condition usually occurs following
the surgical procedures like removal of the tongue.
parotid gland or resection of the ramus of
mandible, etc. ETIOLOGY OF BELL’S PALSY

GLOSSOPHARYNGEAL NEURALGIA The disease may be precipitated by the following
conditions:
Glossopharyngeal neuralgia manifests itself by • Change in the atmospheric pressure, e. g.
repeated episodes of sharp, shooting type of pain
in the ear, pharynx, tonsillar area and the while flying or diving, etc.
posterior part of the tongue, etc. The intensity of • Malignant tumors of the parotid gland and
pain is almost similar to that of the trigeminal
neuralgia and usually there is a “trigger zone” brain.
present in the tonsillar fossa region. • Stroke.
• Surgical procedures in the parotid region
The pain occurs unilaterally, lasts for few • Infections, e. g. acute otitis media and Herpes
seconds to few minutes and the condition mostly
affects the older individuals. Pulse is often slow simplex virus infection.
and fainting may occur if the pain is severe. • Melkersson-Rosenthal syndrome
• Meningitis
TRIGGERING FACTORS • Head injury
• Lyme disease
• Chewing • Multiple sclerosis
• Coughing • Exposure to common cold
• Talking • Following incorrect pterygomandibular block
• Swallowing
• Laughing. anesthesia
• Ischemic damage of the facial nerve.

ETIOLOGY CLINICAL FEATURES (FIGS 20.1 AND 20.2)

The exact etiology of this syndrome is not clearly Paralysis of facial nerve causes loss of all or many
known and the probable initiating factors could of the above mentioned functions:

Diseases of the Nerves and Muscles 573

Fig. 20.1: Bell’s palsy-I Fig. 20.2: Bell’s palsy-II

• Bell’s palsy commonly affects the middle-aged • Recurrent attacks of Bell’s palsy can be
females and mostly the condition occurs associated with “Melkersson–Rosenthal
unilaterally. syndrome”, which also have other features
like cheilitis granulomatosa, fissured tongue
• The paralysis is often rapidly progressing and and edema of the face, etc.
it can be either complete or partial.
• On rare occasions Bell’s palsy can affect
• The patient cannot close the eyeon the affected bilaterally.
side due to loss of muscle control which results
in constant watering from the eye. Key points of Bell’s palsy

• As the eye is constantly open, there will be • Bell’s palsy is the paralysis of facial nerve,
conjunctival dryness. Even ulceration may resulting in inability to control the facial muscles
also occur. on the affected side of the face.

• Drooping of the corner of the mouth on the • Patients with this disease cannot close their eye
affected side is often seen in Bell’s palsy. and are unable to raise their eyebrows on the
affected side due to loss of muscle control,
• Saliva may often run from the affected side of
the mouth; which the patient can not control. • The disease results in constant watering from the
eye and gives the patient a typical ‘mask–like’
• The corner of the mouth on the affected side expressionless face.
does not raise during smile and his gives the
• Slurring of speech, inability to blow whistles by
patient a typical ‘mask–like’ expressionless the mouth and difficulty in taking food, etc are
appearance. the other common problems of Bell’s palsy.
• Most of the patients complain of difficulty in
speech (they have a slurred speech), difficulty • The disease commonly affects the middle–aged
in taking foods and there may be even loss of females and it may be precipitated by several
taste sensations. factors such as sudden exposure to cold, surgical
• The patients cannot raise their eyebrows and injury to facial nerve, change in atmospheric
there is no wrinkle formation in their pressure, head injury or meningitis, etc.
forehead.
• Bell’s palsy patients fail to blow whistles by
their mouth when they try.

574 Essentials of Oral Pathology

TREATMENT Factors precipitating the pain in causalgia

There is no specific treatment for Bell’s palsy. • Emotional stress
Administration of histamine or nicotinic acid has • Visual stimulation
been beneficial in some cases. Physiotherapy is • Auditory stimulation
also helpful in some patients. The eye on the • Physical stimulation
involved side has to be protected from infections • Gentle bridge
by using protective glasses, eye drops and • Family arguments.
ointments, etc.
EAGLE’S SYNDROME
CAUSALGIA
The ‘Eagle’s syndrome complex’ was first
Causalgia refers to the burning pain in the area described by Eagle and hence it is called Eagle’s
of previous injury or surgical procedures; which syndrome.
results from damage to the peripheral nerve. In
the oral cavity it can occur following tooth It is characterized by the following features:
extraction. • Elongation of the styloid process or ossifica-

CRITERIA OF CAUSALGIA tion of the styloid ligament causing dysphagia;
and pain in the throat, pharynx and ear, etc.
• Pain is spontaneous, severe and persistent. • Glossodynia
• It should be present for at least 5 weeks. • Headache
• Pain should be felt distal to the proximal nerve • Vague orofacial pain and pain on the side of
the neck.
injury.
Important causes of paresthesia or
TYPES anesthesia of the lip

Causalgia can be of two types—major and • Inferior alveolar nerve block
minor, the difference is in the degree of severity • Injury or fracture of the jaw
of pain. • Acute osteomyelitis
• Malignant or metastatic tumor of mandible
INJURIES THAT CAN CAUSE CAUSALGIA • Exposed mental foramen
• Herpes zoster
• Bullet injury • Multiple sclerosis
• Head injury • Tetany
• Polio
• Stroke DISEASE OF THE MUSCLES
• Myocardial infarction
• Surgical injury GENERALIZED FAMILIAL MUSCULAR
• Injury from a high velocity sharp instrument DYSTROPHY
• Tooth extractions.
• In few cases, causalgia may develop following Generalized familial muscular dystrophy is a
rapidly progressing muscular disease, which
tonsillectomy. predominantly affects the children and most of
the patients are the sons of their carrier mothers.
NATURE OF PAIN IN CAUSALGIA
CLINICAL FEATURES
The pain can be of several types, which are as
follows: burning, tingling, searing, stabbing, • The earliest manifestation of the disease is
crushing and lightening, etc . inability of the child to stand, run or walk even
after attaining a standard age.
The pain arises within a few days to several
weeks after the tooth extraction, although the oral • Most of the kids fall rapidly while making an
wound has already been completely healed-up attempt to stand or walk by themselves and
by that time. Pain in causalgia is most commonly
felt in the limbs.

Diseases of the Nerves and Muscles 575

this happens due to the weakness of the • Dropping of head, diplopia, ptosis, weight loss
muscles of extremities. and exhaustion
• In severe cases, all the muscles of the body
including those of the facial, masticatory or • Atypical facial pain, candidiasis
ocular groups become involved. • Hyperplasia of thyroid gland
• In generalized familial muscular dystrophy, • Many patients die of respiratory failure but
most of the patients die by the age of 20 years
due to pulmonary infections related to the few patients survive and lead a relatively
respiratory muscle weaknesses. normal life.
Abnormal conditions with which myasthenia
ORAL MANIFESTATIONS gravis is often associated are
• Systemic lupus erythematosus
• Malocclusion • Sjogren’s syndrome
• Open-bite or cross-bite • Progressive systemic sclerosis
• Macroglossia
• Expanded dental arch. TREATMENT
Intramuscular administrations of physostigmine
HISTOPATHOLOGY improve the strength of the affected muscles
readily, but the remission is temporary.
Histologically, the involved muscles reveal
gradual loss of muscle fibers, which are replaced MYOSITIS OSSIFICANS
by connective tissue or fat.
Myositis ossificans is a skeletal muscle disease
LABORATORY DIAGNOSIS of unknown etiology, which is characterized by
the formation of bone (calcification) and
Elevation of serum creatine kinase is a significant connective tissues within the muscles.
laboratory finding.
TYPES
TREATMENT Non-hereditary type—This is the common type,
in which ossification occurs following injury.
There is no satisfactory treatment for this disease. Hereditary type—It is known as myositis ossifi-
cans progressive and in this type the ossification
MYASTHENIA GRAVIS occurs without injury.

Myasthenia gravis is one of the best known Ossifications of the muscle may occur
autoimmune disease in which antibodies are following an inflammatory process and the
produced against acetylcholine receptors of the disease can involve either a single muscle (focal)
muscle end plate of the neuromuscular junctions. or the entire group of muscles (generalized).
This results in an impairment of acetylcholine
signal transmissions across the neuromuscular CLINICAL FEATURES
junction there by causing muscle weaknesses and • Myositis ossificans occur frequently among
pronounced fatigability.
children and young adults.
CLINICAL FEATURES • Most patients develop a single or multiple,

• This disease commonly affects middle-aged soft, fluctuant or firm, nodular, painless
females. swellings on the body, which often arise
following trauma to the affected area.
• The patients often exhibit difficulty in mastica- • In the later stages, some of the nodules
tion and deglutition disappear while the remaining nodules
become bony hard and they often exhibit an
• Dropping of the jaw, slurring of speech, loss overlying red skin.
of taste sensation • The condition can be painful especially when
the affected muscle is used for any work.
• Dry mouth alongwith ulceration of the tongue,
buccal mucosa and palate

576 Essentials of Oral Pathology

• In the orofacial region the masseter muscle is 13. Hill CL, Zhang Y, Sigurgeirsson B. Frequency of
often affected by this disease, which results specific cancer types in dermatomyositis and
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17. Kemler MA, Rijks CP, De Vet HC. Which patients with
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interpretations.

VITAMIN DEFICIENCIES Important oral manifestations

Name of the disease Hyperkeratosis of oral epithelium, decreased salivary flow and dryness
Vitamin A deficiency of mouth.
Hypervitaminosis A
Vitamin B1 (Thiamine) deficiency Cortical thickening of bone, mucosal pigmentations, hemorrhages,
Niacin deficiency retardation of growth.
Riboflavin deficiency
Edema of the tongue, loss of papillae, pain and paresthesia.
Nicotinamide deficiency
Vitamin B12 deficiency Stomatitis and bleeding in the oral mucosa, glossitis and Sandwith’s
Pyridoxine deficiency bald tongue (red, smooth and raw tongue).
Folic acid deficiency
Vitamin C deficiency Reddening and inflammation of tongue with cyanosis and ulceration
(magenta glossitis), angular cheilitis, sore throat, swelling and
Vitamin D deficiency erythema of oral mucosa.

Vitamin K deficiency Glossitis, stomatitis and gingivitis.

Glossitis, aphthous ulcer.

Cheilitis and glossitis.

Glossitis, aphthous ulcer.

Widespread petechiae and ecchymosis of the oral mucosa along with
hyperemia, edema and ulceration. Generalized enlargement of
gingiva with spontaneous bleeding. Severe periodontal inflammation,
alveolar bone loss, tooth mobility and premature loss of teeth. Failure
of wound healing, breaking down of recent wounds and painful
subperiosteal hemorrhage.

Delayed tooth eruption, malpositioning of teeth, retardation of
mandibular growth, development of class II malocclusion. Increased
susceptibility to jaw fracture due to minimum injury.

Increased gingival bleeding, lack of blood coagulation after surgery.