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Published by alysonr, 2018-02-28 08:47:55

HidradenitisSuppurativa PS_080417_Final

HidradenitisSuppurativa PS_080417_Final

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Objectives
Severity Assessments
Treatment ► Review the description, epidemiology, and risk factors
Self-test of HS
Abbreviations
Glossary ► Review the pathophysiology, disease course, and
References signs and symptoms of HS and discuss the
comorbidities associated with HS

► Review the diagnosis and disease severity
assessments of HS

► Discuss the treatment and management for HS

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Description Epidemiology Risk factors
Severity Assessments
Treatment Introduction to HS
Self-test
Abbreviations ► Hidradenitis suppurativa (HS) is a chronic, inflammatory, recurrent, and debilitating skin disease that
Glossary has an estimated worldwide prevalence of 0.05 - 1%.1
References
► Patients develop painful, deep-seated inflammatory nodules in apocrine gland–bearing areas of the skin
that may progress to the formation of abscesses and fistulas.1

► In addition to the physical impact of HS , some patients may experience a substantial impact on health-
related quality of life (HRQoL).1,2

► The disease typically begins after puberty and is associated with several genetic risk factors.1

► Cigarette smoking and obesity may also contribute to the multifactorial cause of HS.3

Formation of microcomedone, comedone, inflammatory abscess, and inflammatory nodule in HS4

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Description Epidemiology Risk factors
Severity Assessments
Treatment Description of HS
Self-test
Abbreviations ► HS is a chronic, recurrent, and debilitating inflammatory skin condition characterized by painful,
Glossary deep-seated inflamed lesions in areas of the skin that have apocrine (sweat or milk) glands.1
References
► Lesions typically appear after puberty, most commonly in intertriginous areas, such as the armpit, groin,
or anal region.1

► Some patients with HS have lesions in the breast, buttock, or pubic regions.3
► The deep-seated inflammatory nodules that characterize HS may initially look like boils or pimples, but the

nodules arise from the dermis (the deepest layer of skin) and are associated with hair follicles.1,3,5,6
► HS was previously referred to as acne inversa.1

HS involvement on the armpit, under the breasts, and on the buttocks2,5,7 Continued
on Next
This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community. Page
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Description Epidemiology Risk factors
Severity Assessments
Treatment Description of HS
Self-test
Abbreviations ► The deep-seated inflammatory nodules can become abscesses
Glossary that spontaneously rupture through the skin surface, draining pus
References that is associated with an unpleasant odor.8

► In severe cases, abscesses that form deep within the dermis may Appearance of Severe HS-Associated
drain through abnormal channels that form and connect to the Scarring in Armpit Area9
surface of the skin. These channels are called sinus tracts or
fistulas.8

► Repeated healing and recurrences of fistulas can lead to
progressive tissue scarring that ultimately takes on a thick,
cordlike band appearance.8

► In some patients, the scarring may be associated with impaired
mobility. For example, scarring under the arm or in the groin area
may make it difficult for a patient to move her arm or walk.5

► In addition to the physical impact of HS , some patients may experience a substantial impact on health-
related quality of life (HRQoL).1,2

► HS can be extremely painful and pain is the most significant factor contributing to impaired HRQoL for
some patients.2

► The skin lesions, particularly draining, malodorous lesions, may lead to embarrassment, disabling social
stigma, low self-worth, and a negative impact on personal relationships.2

► Both physical and psychosocial aspects of HS also may be associated with impaired work productivity.2

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Description Epidemiology Risk factors
Severity Assessments
Treatment Epidemiology of HS
Self-test
Abbreviations Prevalence of HS
Glossary ► Estimated to be 1% worldwide, with higher estimates in European countries compared with the
References
United States.1
► The most recent prevalence estimate available in the United States was 0.05%.1
► The difference between prevalence rates in the United States and Europe may be due to actual differences

in disease prevalence or to only the most severe cases being reported in the United States.1
► For comparison, the prevalence of psoriasis (plaque psoriasis and other types of psoriasis) is

approximately 2% in Europe and North America.10

Age of Onset
► The typical age of onset of HS is in the early 20s and rarely occurs before puberty or after age 50.7
► If HS appears before age 11, it is usually because of early puberty.5
► The disease tends to be most active in patients in their 20s and 30s.2

Sex Differences
► More women are affected by HS than men, in a 3:1 ratio.7
► Compared with men, women tend to have more lesions in the inguinal (groin) area (the junction between

the legs and the pelvic area ), and some women with HS have lesions below the breasts.2,6,7
► Men tend to have lesions in the perineal/perianal and buttock regions, or in atypical areas, such as the ears

and chest.2

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Description Epidemiology Risk factors
Severity Assessments
Treatment The etiology of HS is likely multifactorial3
Self-test
Abbreviations Genetic Risk Factors
Glossary
References ► Thirty-four percent of first-degree relatives of HS patients also suffered from HS, according to a study reported in 1985. This
finding indicated that HS is transmitted as an autosomal dominant trait.1

► Recent studies also indicate that 30% to 40% of patients with HS report a family history of HS.1

► The specific genetic mutations underlying HS have not yet been identified, with one exception. In about 5% of patients with HS,
various mutations in the gamma-secretase gene have been identified. Patients with these mutations, which have been identified in
British, Chinese, and French families, have a particularly severe form of HS.1

► Other gene mutations that may predispose individuals to HS and/or influence disease severity are currently being investigated.1

Overweight and Obesity as Risk Factors Environmental Risk Factors

► Being overweight or obese is a well-established risk factor for ► Tobacco: Cigarette smoking is another well-established risk
HS and may also contribute to disease severity.1 factor for HS.1,3,11

o Being overweight, defined as having a body mass index o In one study in a French population, current smokers were
(BMI) of 25 kg/m2 to 30 kg/m2, doubles the risk of HS.1 12 times more likely to have HS than nonsmokers.1,3,11

o Being obese, defined as a BMI ≥ 30 kg/m2 quadruples the o Several studies indicate that approximately 70% to 90% of
risk of HS. Greater BMI has also been associated with patients with HS smoke cigarettes.1,3,11
greater disease severity in patients with HS.1
► Mechanical stress: Mechanical stress has been proposed
► In patients with HS, body weight may affect skin physiology as a trigger for new HS lesions but a causal link has not yet
and also contribute to disease severity via mechanical been established.1
effects.3
o Some healthcare practitioners have proposed mechanical
o For example, being overweight or obese may be associated stress as a trigger based on the role of obesity as both a risk
with increased sweat retention, maceration, and enhanced factor for HS and a contributing factor to disease severity.1
mechanical friction of opposed skin surfaces.3
o In addition, patients with HS report that tight clothing can
o In addition, some patients who are obese may have trigger an outbreak of lesions.1
hormonal alterations that contribute to HS severity.3

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Pathophysiology Disease Course Signs and Symptoms Comorbid Disorders
Severity Assessments
Treatment Overview of pathophysiology
Self-test
Abbreviations ► HS is a chronic immune-mediated inflammatory disorder that appears to begin with hyperkeratosis of
Glossary hair follicles in apocrine gland-bearing skin.1,12
References
► The hair follicle becomes plugged with keratinocytes, and rupture of the hair follicle leads to a massive,
local immune response that may lead to inflammation, abscess formation, and in later stages, fistula
formation and scarring.1,12

► HS has a chronic course that is marked by intermittent periods of activity and remission.3

► Patients with HS also are at an increased risk for developing several comorbid chronic diseases,
particularly other inflammatory disorders.1

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Pathophysiology Disease Course Signs and Symptoms Comorbid Disorders
Severity Assessments
Treatment Pathophysiology of HS
Self-test
Abbreviations ► The pathophysiology of HS likely involves a complex interplay of multiple genetic, immunologic, and
Glossary environmental factors.1,3
References
► Few data are available about the precise mechanism leading to tissue inflammation and damage in HS.1
► However, the central pathologic event is believed to be increased production of keratinocytes within the

hair follicle (hyperkeratosis) that leads to occlusion (plugging) of the upper parts of the hair follicle and
subsequent rupture.1,12
► Rupture of the hair follicle leads to a massive, local immune response, resulting in inflammation, abscess
formation, and in later stages, fistula formation and scarring.12

Typical morphological features include inflamed nodules, abscesses, sinus tracts, and hypertrophic fibrous scarring.3

Continued
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Page

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Pathophysiology Disease Course Signs and Symptoms Comorbid Disorders
Severity Assessments
Treatment Pathophysiology of HS
Self-test
Abbreviations Immunopathogenesis
Glossary ► In early HS lesions, the inflammatory process, including abscess formation, is associated with an influx of white
References
blood cells (WBCs), primarily macrophages, monocytes, and dendritic cells.1
► Because the abscesses are associated with WBCs, they are called neutrophilic abscesses.1
► The macrophages that are found in HS lesions release the proinflammatory cytokines: interleukin 12 (IL-12) and

IL-23. IL-23 is associated with activation of T helper cells (specifically Th17 cells) that release IL-17, which is a
proinflammatory cytokine. Th17 cells also have been found in HS lesions.1
► In addition, increased levels of tumor necrosis factor (TNF) have been identified in HS lesions, along with
increased levels of IL-10, an anti-inflammatory cytokine.3
► The inflammatory reactions leading to HS show many similarities with the inflammatory reactions in plaque
psoriasis.1

Gamma-secretase Gene Mutations Infection
► Approximately 5% of patients with HS have a defect in ► Bacterial infection is not thought to be the primary

the gamma-secretase gene that is associated with cause of HS.3
severe diseases.1 ► However, plugged hair follicles may provide an area for
► In these patients, genetic mutations in the gamma-
secretase–Notch signaling pathway may lead to bacteria to grow, thus triggering an immune response.3
abnormal epithelial proliferation and differentiation and ► It has also been proposed that normal bacteria in or
result in formation of epidermal cysts that are enriched
with follicular keratin.1,3,12 near hair follicles may trigger an abnormal immune
response.3

Apocrine Glands
► Although the inflammation associated with HS does not appear to originate in the apocrine (sweat or milk) glands,

the fact that HS lesions occur only in areas of skin that contains apocrine glands indicates that there may be an
apocrine effect on HS pathogenesis.1

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Pathophysiology Disease Course Signs and Symptoms Comorbid Disorders
Severity Assessments
Treatment Disease Course
Self-test
Abbreviations ► HS is a chronic, relapsing, inflammatory skin condition that typically starts between In its earliest stage, HS often looks
Glossary puberty and age 40 , often in people in their early 20s.2,7,13 like boils or pimples5
References
► The prevalence of HS declines after 50 years of age.7 In later stages of HS, thick, painful
► Patients may have a single, painful bump that persists for weeks or months or may scars can form.5

experience breakouts of lesions that clear and reappear.5,13
► Some patients repeatedly experience lesions in the same spot on their skin, and

some experience breakouts in the same general area of their skin but not in the
exact spot.5
► The signs and symptoms of HS can change quickly. One week, a patient may suffer
from nodules that are leaking a foul-smelling liquid. The next week, the patient’s
lesions may have cleared, leaving scars as the only remaining sign of the disease.5
► Some patients experience only mild disease, but for other patients, HS may
progressively worsen and affect multiple areas of their body.13
► Nodules that form deep within the dermis may become pus-filled cavities
(abscesses) that can spontaneously rupture and leak through the skin. The draining
fluid often has an unpleasant odor.8
► In severe cases of HS, the abscesses may drain via abnormal channels that form
deep within the dermis and connect the abscesses to the skin surface.8 These
tunnel-like channels are called fistulas. The skin over these areas becomes
compromised and may develop a sponge-like appearance.3,5
► If lesions repeatedly heal and reappear in the same area, scars may form. As
mentioned previously, these scars may thicken over time, taking on a thick, rope-like
appearance.8
► Patients with long-standing, poorly controlled HS that is associated with substantial
scarring may experience impaired mobility.
o For example, this type of impairment may occur in patients who have lesions in

the armpit.3 Scarring in the armpit may make moving the arm difficult.5

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Pathophysiology Disease Course Signs and Symptoms Comorbid Disorders
Severity Assessments
Treatment Common locations and appearance of HS lesions
Self-test
Abbreviations ► As previously noted, HS occurs in areas of the
Glossary skin that contain apocrine glands, which are
References the oil and sweat glands around hair follicles.1

► The most commonly affected areas of the skin
are the armpits, groin, and anal area.1

► HS may also occur in other areas where skin
rubs together (intertriginous areas), such as
under the breasts in women or between the
buttocks.13

► In women, lesions on the groin, thigh, and
breast areas are also common, whereas in
men, lesions on the buttocks, in the
perineal/perianal regions, and in atypical areas,
such as the ears and chest, are also common.2

Common affected areas and appearance of HS14

Continued
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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Pathophysiology Disease Course Signs and Symptoms Comorbid Disorders
Severity Assessments
Treatment Physical Signs and Symptoms
Self-test
Abbreviations Signs and symptoms of HS include2,7,8,13
Glossary
References Blackheads small, pitted areas of skin that often appear in pairs are a common feature

Red, tender bumps bumps that look like boils are the hallmark sign of HS
► Appear more rounded in shape and are located deeper in the dermis than boils
► May enlarge, rupture, and drain pus that has an unpleasant odor (ie, abscess formation)
► May be accompanied by itching and burning
► Appear in areas where skin rubs against skin (typically)

Painful, pea-sized lumps hard lumps (inflammatory nodules) that develop under the skin
► May persist for years
► May enlarge and become inflamed

Leaking bumps or sores Leaking bumps or sores—open wounds that heal slowly (if at all)
► May lead to scar formation where lesions have repeatedly healed and reappeared
► May lead to development of tunnel-like channels called fistulas deep in the dermis

Pain1,2

► Pain may be one of the most important symptoms reported by patients.
o The European S1 guideline notes that patients describe the pain using such terms as hot, burning, stretching, cutting, sharp, taut,
splitting, throbbing, and aching.

► The pain associated with HS may be intense and chronic, and is typically linked to the deep-seated inflammatory nodules.
► Some patients report that HS-related pain is the most significant factor contributing to an impaired HRQoL.

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Pathophysiology Disease Course Signs and Symptoms Comorbid Disorders
Severity Assessments
Treatment HS may be associated with a variety of comorbid disorders1,15,16
Self-test
Abbreviations Common Comorbid Disorders
Glossary The most common comorbidities in patients with HS are1:
References
○Follicular occlusion syndrome ○ Inflammatory bowel disease (IBD) ○Spondyloarthritis
► Follicular occlusion syndrome includes disorders that, like HS, involve occlusion of hair follicles. These

disorders are acne conglobata, dissecting cellulitis of the scalp, and pilonidal cysts.15
► Like HS, IBD and spondyloarthritis are chronic, immune-mediated inflammatory diseases.1

Metabolic Syndrome
► As in patients with plaque psoriasis, patients with HS are significantly more likely than their healthy

counterparts to have metabolic syndrome.1,16
► Metabolic syndrome is a chronic inflammatory state that is associated with a substantially increased risk of

cardiovascular mortality.16
► The presence of at least 3 of the following 5 criteria defines the metabolic syndrome16:

○ Increased waist circumference or abdominal obesity
○ Hypertension (high blood pressure)
○ Hypertriglyceridemia (high levels of fat in the blood)
○ Reduced high-density lipoprotein (HDL) levels (ie, reduced good cholesterol levels)
○ Insulin resistance

Continued
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This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Pathophysiology Disease Course Signs and Symptoms Comorbid Disorders
Severity Assessments
Treatment HS may be associated with a variety of comorbid disorders1,14,15
Self-test
Abbreviations Psychosocial Disorders
Glossary ► HS may have a significant emotional impact on patients.1
References ► In a study using the Dermatology Life Quality Index (DLQI), which is a patient-reported outcome (PRO)

measure, patients with HS had a greater impairment in HRQoL than patients with other dermatologic conditions
such as plaque psoriasis.1
► Although clothing can cover visible signs of the disease, such as active lesions and scarring, discharge from active
lesions may have an odor and also stain clothing.1,2
► The physical manifestations of HS may lead to embarrassment, isolation due to social stigmatization, and low self-
worth.1,2
► Patients with HS may also experience a negative impact on interpersonal relationships, including significantly
impaired sexual health compared with age-, sex- and BMI-matched individuals.1,2
► Approximately 1 in 5 patients with HS is diagnosed with depression.1

Associated with Severe or Long-Standing Disease
► For patients with severe HS, the overall burden of disease may be compounded by concomitant pyoderma

gangrenosum and complications of severe HS, such as anemia, amyloidosis, and lymphedema.1,2
► Pyoderma gangrenosum is an uncommon inflammatory skin condition associated with large, painful ulcers that

typically occur on the legs.
o This condition and HS may share a common dysregulated cytokine profile.2
► Patients with long-standing HS have an increased risk for epithelial and nonmelanoma skin cancers.2
o For example, squamous cell carcinoma (cancer of squamous cells in the skin ) has been documented particularly

in men with HS who have had lesions for 10 to 30 years.1,17

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Diagnosis
Severity Assessments
Treatment Diagnosis of HS
Self-test
Abbreviations ► The diagnosis of HS is made clinically, based on
Glossary the results of a medical history, family history, and
References physical examination.1,3

► The physician also may consider information
obtained from laboratory and microbiology tests
when making the diagnosis, but there is no single
test to diagnose HS.1,3

► Physicians rule out diseases that may cause similar
signs and symptoms using a differential diagnosis.7

► Imaging studies, such as ultrasound, are only
used for preoperative assessment in patients with
HS who require surgical intervention.7

► The median delay from onset of HS to diagnosis is
approximately 7 years.19

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Diagnosis
Severity Assessments
Treatment Diagnosis of HS
Self-test
Abbreviations Medical History
Glossary
References ► The physician, typically a dermatologist, will ask the patient about symptoms and the frequency,
recurrence, and duration of lesions.5

► A history of recurrent painful or draining lesions more than twice in a 6-month period may indicate HS.1
► Also, a family history of HS may support a diagnosis of HS.1

Laboratory Assessments

► A physician may order blood tests as part of the diagnostic process in a patient with HS.
► Laboratory measurements are typically normal in patients with HS; however, patients with severe HS may

have elevations in the following measurements:3
o WBCs
o Erythrocyte sedimentation rate (ESR)
o C-reactive protein (CRP)
► Bacterial cultures of skin swabs or draining lesions may be obtained to rule out an infectious cause of the
skin lesions. HS is not caused by bacteria.1,3,5,7

Continued
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Page

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Diagnosis
Severity Assessments
Treatment Diagnosis of HS
Self-test
Abbreviations Physical Examination
Glossary
References ► Patients with HS typically appear clinically well and do not have Abscess/ nodule formation HS affected breast region2
an elevated body temperature, even patients with severe (Initial Stage)9
disease.3
Multiple interconnected tracts and abscesses in severe HS7,9
► HS is associated with the presence of characteristic inflamed and
noninflamed nodules that appear deeper in the dermis than
simple boils.7

► Depending on disease severity, HS may also be characterized by
the presence of abscesses, draining and nondraining fistulas,
plaque-like induration, and ropelike scarring.7

► The physician will determine the presence of lesions in areas
typically affected by HS, such as the armpit, groin, or breasts
in women.1,7

► Patients may have single lesions or multiple inflamed lesions. In
patients with multiple lesions, the lesions (such as abscesses or
fistulas) may be widely separated within a region of the skin.1

► Alternatively, the lesions may merge in more severe cases.1

► Skin biopsies are generally only used to evaluate disease in
atypical or refractory cases of HS.7

Differential Diagnosis

In the process of making a diagnosis of HS, the physician rules out other potential causes of inflammatory nodules or draining
skin lesions. Other conditions that may share some signs and symptoms with HS include1,3:
○Simple abscesses ○Staphylococcal or other infections ○Cutaneous Crohn’s disease ○Certain types of neoplasms

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Disease Severity Classification Disease Severity Risk Factors
Severity Assessments
Treatment Disease Severity Assessments Overview
Self-test
Abbreviations ► HS disease severity is evaluated during initial diagnosis and also throughout the disease course.1,18
Glossary
References ► Clinicians may use several methods to assess disease severity, both in clinical practice and in
clinical trials.18

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Disease Severity Classification Disease Severity Risk Factors
Severity Assessments
Treatment A number of HS disease severity classification systems have been developed
Self-test
Abbreviations Hurley Staging System
Glossary
References ► The first was the Hurley staging system, proposed in 1989, and this system of classifying HS is generally still used to
determine disease severity.1,7

► The Hurley staging system classifies HS into 3 stages of disease severity based on clinical features.1

Hurley Staging System1,3

Hurley Clinical Features % of
Stage Patients
Affected

Stage 1 Abscess formation (single or multiple) 68%
(mild) without fistulas or scar formation

Stage 2 Recurrent abscesses (single or 28%
(moderate) multiple) with fistulas or scar formation.
Lesions are distinct and widely
separated (eg, > 10 cm apart)

Stage 3 Multiple interconnected fistulas and/or 4%
(severe) abscesses spread across an entire
area of the body (eg, axilla)
Appearance of HS by Hurley Stage Disease Severity

► While useful for rapid classification of HS severity, the Hurley staging system has limitations.1,18 Continued
o For example, the Hurley staging system only measures static disease characteristics, such as the presence of on Next
scarring and fistulas that are unlikely to change during medication treatment.1,18 Page
o Also, this staging system does not measure inflammatory features, such as erythema and discharge.1,18

► Therefore, the Hurley staging system is not suitable as an efficacy measurement in clinical trials.1,18

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Disease Severity Classification Disease Severity Risk Factors
Severity Assessments
Treatment A number of HS disease severity classification systems have been developed
Self-test
Abbreviations HS-Physician Global Assessment1,9,20 Modified Sartorius Score1,9,18
Glossary
References ► The HS-PGA is another tool that is used to measure HS ► The original Sartorius score was developed to
disease severity. The HS-PGA has 6 categories represented provide a more detailed assessment of
on a scale of 0 to 5: disease severity compared with the Hurley
staging system.
Score Description
Clear (0) no inflammatory or noninflammatory nodules ► The modified Sartorius score involves:

Minimal (1) only the presence of noninflammatory nodules o counting the number of involved regions
Mild (2)
Moderate (3) less than 5 inflammatory nodules or 1 abscess or o counting the number and type of involved
draining fistula and no inflammatory nodules lesions
Severe (4)
Very severe (5) less than 5 inflammatory nodules or 1 abscess or o measuring the longest distance between
draining fistula and 1 or more inflammatory nodules 2 lesions
or 2 to 5 abscesses or draining fistulas and less than
10 inflammatory nodules o adding extra points to Hurley stage 3 areas.

2 to 5 abscesses or draining fistulas and 10 or more
inflammatory nodules

5 or more abscesses or draining fistulas

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Disease Severity Classification Disease Severity Risk Factors
Severity Assessments
Treatment Risk factors for Greater Disease Severity
Self-test
Abbreviations ► As previously noted, greater BMI is associated with greater disease severity in patients with HS.1
Glossary ► Additional risk factors for greater disease severity include:3
References
o Disease duration > 15 years
o Male sex
o Smoking > 15 pack-year history
o Presence of lesions in the armpit, breast, or perianal regions
o Presence of atypical locations (eg, ears)
o Personal history of severe acne
o No family history of HS
► In addition, stress, hormonal changes, and heat or humidity may also worsen symptoms. In women,
disease severity may lessen after menopause occurs.13

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Hidradenitis Suppurativa Treatment Overview
Self-test
Abbreviations ► HS is a chronic disease with no cure.5
Glossary
References ► Treatment may help patients with HS clear or reduce lesions, get rid of scars and tunnels beneath the skin,
and prevent new lesions.5

► The treatment approach for a patient with HS may be individualized depending on many patient factors.3,21

► Treatment options for HS include lifestyle management, topical and systemic medication therapy, and
surgical therapy.1

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Treatment Approach
Self-test
Abbreviations ► HS is a chronic disease with no cure. A comprehensive approach to HS treatment requires assessment of
Glossary disease severity and consideration of the following factors:8,21
References
o Controlling inflammation within and surrounding the follicle
o Preventing complications
o Slowing progression to Hurley stage 3 for as long as possible

► The European S1 guideline for the treatment of HS recommends that HS be treated based both on
subjective impact on the patient and objective disease severity.1

► Treatment may help patients with HS clear or reduce lesions, get rid of scars and tunnels beneath the skin,
and prevent new lesions.5

► Currently, there are no medications, including Amgen medications, that meet all of the goals of moderate to
severe HS treatment. HS remains a difficult to treat disease in spite of the progress of medication
development in recent years that have altered the treatment landscape and impacted patients.1,3

When evaluating treatments and speaking to customers about Amgen products, you must only
discuss the specific proven effects as described in the FDA-approved labeling and company-
approved promotional materials.

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Treatment Approach
Self-test
Abbreviations ► Depending on disease severity and the type of An individualized treatment approach
Glossary treatment needed, patients with HS may obtain for HS is based on a number of
References care from a primary care practitioner, a factors3,21:
dermatologist, and/or a surgeon.3
► Individual lesion type
► Treatment options for HS include1:
► Disease severity
o Lifestyle modifications
o Medication treatment ► Patient’s response to past and
o Surgical interventions current treatment(s)

► Tolerability of therapies

► Potential for adverse reactions

► Comorbidities

No single treatment will work for every patient with HS. A patient may need to try a few different treatments to
find one that works.5

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Lifestyle Modifications | Medication Treatment | Surgical Treatment
Self-test
Abbreviations Lifestyle Modifications
Glossary
References ► Lifestyle modifications for patients with HS may include weight loss, smoking cessation, and avoidance of
tight clothing.1,3,5

► Weight loss for patients with HS who are overweight or obese may be particularly helpful in managing HS.5

o In one retrospective study of patients who had undergone bariatric surgery, a >15% reduction in body
weight was associated with a 35% reduction in HS symptoms.3

► Because of the association between cigarette smoking and HS, patients who smoke are encouraged to quit
smoking.1

► In addition, it is recommended that patients with HS avoid tight-fitting, restrictive clothing, given that friction
may trigger or worsen HS lesions.3

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Lifestyle Modifications | Medication Treatment | Surgical Treatment
Self-test
Abbreviations Medication Treatment
Glossary
References ► The medication choice for an individual patient with HS depends on disease severity.7

o Patients with localized lesions (Hurley stage 1) may be treated with topical medication therapy

o Patients with more widespread or severe disease (Hurley stage 2 or 3) may require systemic
medication therapy.7

o Patients with moderate or severe disease may be treated with a combination of topical and systemic
therapy.3

► Data from clinical trials of medications for HS are limited, so physicians typically rely on clinical
experience.7

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Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Lifestyle Modifications | Medication Treatment | Surgical Treatment
Self-test
Abbreviations Medication Treatment
Glossary
References Topical Medications

► Topical agents that may be used to treat patients with HS include exfoliants and topical antibiotics.1
o An exfoliant may help eliminate excess keratinocytes, reduce itching, and have antibacterial properties.1

► In addition, as previously noted, pain is a substantial contributor to the overall morbidity associated with HS.1
► Topical analgesic medications, therefore, may be used to manage the pain associated with HS.3

Systemic Medications

► Systemic medications may be used in patients with moderate to severe HS.1
Options for systemic treatment include nonbiologic and biologic medications.1

Nonbiologic agents
o Nonbiologic medications that may be used systemically to treat patients with HS include antibiotics, anti-inflammatory
agents, and hormones.1
o The antibiotics used to treat HS are typically those with anti-inflammatory and immunomodulatory properties.7
o In addition, systemic analgesics may be used for pain management.1,3
o However, as of 2016, there were no nonbiologic medications that were approved by the FDA for the treatment of HS.22,23

Biologic agents
o Biologic medications, including biosimilars, are another form of systemic therapy for moderate to severe HS.1
o A physician may select a biologic medication to treat patients with moderate to severe HS whose lesions have not
responded to treatment with nonbiologic systemic agents.3
o Humira® (adalimumab), a TNF blocker administered by subcutaneous injection, is the first medication approved by the
FDA for the treatment of moderate to severe HS.5,24
o As of 2016, adalimumab was the only medication with an FDA-approved and European Medicines Agency-approved
indication for moderate to severe HS.22,23

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Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Lifestyle Modifications | Medication Treatment | Surgical Treatment
Self-test
Abbreviations Medication Treatment
Glossary
References ► Adalimumab is indicated for the treatment of moderate to severe HS . In clinical trials of patients with moderate to severe HS,
adalimumab was associated with a reduction in total abscess and inflammatory nodule count.24

The prescribing information (PI) for adalimumab has boxed warnings for:24

o Serious infections that may lead to hospitalization or death; reported infections include tuberculosis; invasive fungal
infections; and bacterial, viral and other infections due to opportunistic pathogens

o Lymphoma and other malignancies, some fatal, have been reported in children and adolescent patients treated with TNF
blockers.

► In addition to the boxed warnings, it is important to understand any The PI for adalimumab has Continued
contraindications, warnings and precautions, and the most common adverse warnings and precautions for:24 on Next
reactions for adalimumab. ► Serious infections Page
► Malignancies
o There are no contraindications listed in the PI for adalimumab.24 ► Hypersensitivity reactions
► Hepatitis B virus reactivation
o The most common adverse events (> 10%) for adalimumab are: ► Neurologic reactions
infections (eg, upper respiratory, sinusitis), injection-site reactions, ► Use with anakinra
headache, and rash.24 ► Heart failure
► Autoimmunity
► Key proinflammatory cytokines in the HS disease process include IL-12, ► Immunizations
IL-17, IL-23, and TNF.1,3 ► Use with abatacept

► Adalimumab is a recombinant human immunoglobulin G1 monoclonal
antibody (mAb) specific for human TNF.
TNF blockers like adalimumab may also be referred to as TNF inhibitors or
TNF antagonists.24

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Lifestyle Modifications | Medication Treatment | Surgical Treatment
Self-test
Abbreviations Surgical Treatment
Glossary
References ► Surgical treatment is a common and accepted therapeutic option for patients with HS, given that
nonsurgical treatment options seldom result in a lasting cure. Several surgical treatment methods are
used to treat patients with HS, including1:

o Conventional surgery

o Deroofing

o Carbon dioxide laser therapy

o Nd:YAG laser therapy

► All surgical treatments for HS are intended to remove all keratinocytes in nodules, abscesses, and
fistulas.1 Removing the keratinocytes removes the pathologic process. This removal can be done through
excision of whole or parts of an involved area of the skin.1

► The type of surgical treatment selected and the extent of surgical intervention depend on HS disease
severity and the region of the body affected.1

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Lifestyle Modifications | Medication Treatment | Surgical Treatment
Self-test
Abbreviations Surgical Treatment
Glossary
References Conventional Surgery

► Complete (sometimes called wide or radical) excision of an apocrine gland–bearing area of skin that is affected by
HS may be the recommended surgical treatment for some patients.1

► Following removal of the affected skin and underlying tissue, the wound may be allowed to heal on its own without
any type of reconstructive surgery.1

► In other cases, various types of reconstructive surgery, such as the use of skin grafting techniques, may be used.1

○ Deroofing

► involves removing the top of an HS lesion by using an electrosurgical loop. This procedure is typically used in
patients with HS who have Hurley stage 1 or 2 disease with recurrent lesions in fixed locations. Following the
procedure, the surgically treated areas are left open to heal.1

► The deroofing technique converts painful recurrent HS lesions into cosmetically acceptable scars . The European
S1 guideline notes that the procedure is fast and effective and may be accomplished in a physician’s office.1

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Lifestyle Modifications | Medication Treatment | Surgical Treatment
Self-test
Abbreviations Surgical Treatment
Glossary
References Carbon dioxide laser therapy

► Carbon dioxide laser treatment allows focal radical vaporization of all nodules, abscesses, and fistulas in an affected
region of the skin.1

► The lesions are vaporized from ‘inside and out’ until healthy tissue surrounding the HS lesion is reached.1

► The carbon dioxide laser can be used to remove superficial lesions and also to remove deep lesions. In addition,
this type of therapy can be targeted on small or larger areas of affected skin.1

► Treated areas are usually left open to heal1

○Nd:YAG laser therapy

► neodymium-doped yttrium aluminum garnet (Nd:YAG) laser was originally designed for hair removal.1

► Because the pathophysiology of HS involves the hair follicle, the rationale for using Nd:YAG laser treatment to
destroy the hair follicle is applicable to patients with HS.1,5

► Hair removal using the Nd:YAG laser has been shown to decrease the severity of HS, though it is not yet
established as a standard treatment for HS.1

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Assessing Treatment Response in HS
Self-test
Abbreviations ► Although the Hurley staging system has been used for many years to assess HS disease severity, it was
Glossary not designed as a dynamic measure of treatment response.20
References
► The Modified Sartorius Score and the HS-PGA can be used to measure both HS disease severity (as
described previously) and treatment response.20

► In recent years, novel, validated measures of HS that are responsive to improvement in disease activity,
including the Hidradenitis Suppurativa Clinical Response (HiSCR) and the Hidradenitis Suppurativa
Severity Index (HSSI), have been developed specifically for use in clinical trials of biologic medications.1,20

It is important to note that some of these measures were not used in the clinical development and FDA-
approval for adalimumab. When discussing Amgen products, only measures that were utilized in clinical
studies and included in the approved labeling and/or promotional materials should be discussed.

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Measures of HS Treatment Response: HS-PGA and Modified Sartorius Score
Self-test
Abbreviations HS-PGA1,9 Modified Sartorius Score 1,9,18
Glossary
References ► The HS-PGA, which was described previously ► The original Sartorius score was developed, in part, to
as a tool to measure HS disease severity, is the provide a dynamic measure of clinical severity, which
most frequently used assessment tool to the Hurley staging system does not provide.
measure clinical improvement in clinical trials of
medications for HS. ► As previously noted, the Modified Sartorius score
involves counting the number of involved regions, the
► As previously noted, the HS-PGA has 6 number and type of involved lesions, measuring the
categories represented on a scale of 0 to 5, longest distance between 2 lesions, and adding extra
from clear (no inflammatory or noninflammatory points to Hurley stage 3 areas.
nodules) to very severe (more than 5
abscesses or draining fistulas). ► The Modified Sartorius score is sometimes used in
clinical trials to measure treatment response, though
its use in patients with severe disease is limited when
there is no longer separation between lesions.

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Measures of HS Treatment Response: HiSCR and HSSI
Self-test
Abbreviations Hidradenitis Suppurativa Clinical Hidradenitis Suppurativa Severity Index (HSSI)
Glossary Response (HiSCR)
References ► The Hidradenitis Suppurativa Severity Index (HSSI) is a
► The HiSCR was developed specifically to assess change composite score that assesses 5 aspects of HS30:
in disease severity during medication treatment.18,20
o Number of sites affected (scored as 0-4)
► A HiSCR is defined as a ≥ 50% reduction in
inflammatory (transient) lesion count (sum of abscesses o Percent body surface area (% BSA) affected
and inflammatory nodules) and no increase in abscesses (scored as 0-4)
or draining fistulas (chronic inflamed lesions) compared
with baseline.18,20 o Number of lesions that are erythematous and painful
(scored as 0-4)
► Achievement of a Hidradenitis Suppurativa Clinical
Response (HiSCR) provides a meaningful clinical o Number of dressing changes required for draining lesions
endpoint when studying the inflammatory manifestations per working or leisure hour (scored as 0-3)
of HS.24,29
o Pain (scored as 0-4)
► The sum of abscesses and nodules is sometimes
referred to as an AN count.20 ► The sum of the 5 category scores is then used to obtain a
final score ranging from 0 to 19. The final HSSI score
o Achievement of ≥ 50%, 75%, or 100% reduction in indicates HS disease severity based on the following
AN count may be referred to as an AN50, AN75, or ranges29:
AN100 response, respectively.20
o Mild: 0 to 7
► The 50% threshold for the HiSCR is considered clinically
appropriate and meaningful to the patient and is a more o Moderate: 8 to 12
sensitive measure of change in disease activity than the
HS-PGA or the Modified Sartorius score.20 o Severe: > 13

► The HSSI can be used to measure treatment response in
clinical trials. In clinical trials of biologic medications, a
treatment response is typically defined as a 50% reduction in
HSSI score from baseline to endpoint.30,31

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Treatment Strategies
Self-test
Abbreviations Depending on the severity of HS lesions, monotherapy with a topical or systemic agent may be sufficient, but
Glossary for other patients, combination therapy with medication and surgical interventions may be the appropriate
References treatment strategy.1

► As previously noted, because the cause of HS is multifactorial, no single treatment will be effective for
every patient.21

► Overall, a multimodal approach that includes lifestyle modifications in addition to medical and/or surgical
treatments may be the most effective treatment approach for most patients with HS.3

► The treatment strategy for an individual patient with HS depends on disease severity.1

o Topical medication therapy may be used for patients with mild or moderate HS as monotherapy.1

o It may also be used as part of combination therapy for patients with moderate to severe HS who are
receiving systemic medications.3

o Biologic therapy is typically reserved for patients with moderate to severe HS.1

► Surgery also has a role in the treatment of HS.

o Surgery may be preferred for patients who have locally recurring lesions.1,7

o Medication treatment also may be combined with surgical treatment in patients with severe disease.1

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Overview Approach Response Assessment Strategies Guidelines
Severity Assessments
Treatment Treatment Guidelines
Self-test
Abbreviations The European Academy of Dermatology and Venereology (EADV) published the European S1 guideline for the treatment of HS
Glossary in 2015.1 Before the EADV guidelines were published, no formal guidelines were available for the management of HS.7
References
The European Academy of Dermatology and Venereology (EADV) was founded in 1987 to advance excellence in clinical care,
research, education, and training in the field of dermatology and venereology.32

European S1 Guideline

► The European S1 guideline for the treatment of HS discusses the use of various HS treatments, including lifestyle
management; topical therapies; systemic agents, including nonbiologic and biologic therapies; and surgical therapies.1

► The European S1 guideline for HS recommends a treatment approach that is based on the individual subjective impact and
objective severity of the disease, as measured by the Hurley staging system.1

o All patients with HS may benefit from lifestyle and pain management.1

o Topical medication treatment may be recommended for patients with mild HS, whereas systemic medications may be
recommended for patients with moderate to severe HS.1

o Surgical treatment, such as deroofing, laser treatment, or local excision, may be recommended for locally recurring
lesions, whereas widely spread and/or more severe lesions may require wide surgical excision.1

European S1 Treatment Guideline1

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Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Self Test
Severity Assessments
Treatment Question 1
Self-test
Abbreviations HS lesions most commonly appear in which areas of the skin?
Glossary a) Breast, buttocks, pubic area
References b) Chest, scalp, ear
c) Armpit, groin, anal area
d) Arm, leg, back Continued
on Next
Question 2 Page

HS is characterized by which of the following types of skin lesions? (Select all that apply.)
a) Inflammatory nodules
b) Abscesses
c) Scaly plaques
d) Fistulas

Question 3

True/False? HS is characterized by lesions in areas of the skin that have apocrine glands.
a) True
b) False

Question 4
All of the following statements about the epidemiology of HS are true EXCEPT: (Select all that apply.)

a) The prevalence is estimated to be 1% worldwide
b) The typical age of onset is in the early 20s
c) More women are affected than men, in a 3:1 ratio
d) The onset often occurs after menopause

Question 5
Which of the following statements about risk factors for HS are true? (Select all that apply.)

a) 30% to 40% of patients with HS report a family history of HS
b) Obesity quadruples the risk of HS
c) Viral infections increase the risk of HS
d) Cigarette smoking is a well-established risk factor for HS

Question 6

Which of the following statements about the pathophysiology of HS are true? (Select all that apply.)
a) Bacterial infection is the primary cause of HS .
b) The central pathologic event is hyperkeratosis in the hair follicle that leads to plugging and subsequent rupture .
c) HS is caused by pyoderma gangrenosum.
d) The proinflammatory cytokines IL-12, IL-17, IL-23, and TNF are associated with HS lesions .

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Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Self Test
Severity Assessments
Treatment Question 7
Self-test
Abbreviations All of the following statements accurately describe the disease course of HS EXCEPT:
Glossary a) Patients may have a single, painful bump that persists for weeks or months or may experience breakouts of lesions that clear and reappear.
References b) Nodules that form deep within the dermis may become pus-filled cavities (abscesses) that drain fluid with an unpleasant odor .
c) Severe HS is rarely associated with scarring.
d) Long-standing, poorly controlled HS may be associated with substantial scarring and patients may experience impaired mobility.

Question 8

Which of the following are signs and symptoms of HS? (Select all that apply.)
a) Red, tender bumps that look like boils
b) Painful, pea-sized hard lumps (inflammatory nodules) that develop under the skin
c) Swollen joints
d) Leaking bumps or sores that heal slowly (if at all)

Question 9

True/False? Patients with HS are not significantly more likely than their healthy counterparts to have metabolic syndrome.
a) True
b) False

Question 10

What are the 3 most common comorbidities associated with HS?
a) Pyoderma gangrenosum, spondyloarthritis, and amyloidosis
b) Anemia, amyloidosis, and lymphedema
c) Follicular occlusion syndrome, IBD, spondyloarthritis
d) Plaque psoriasis, IBD, and anemia

Question 11

How is the diagnosis of HS typically made?
a) Histologically, based on the results of a skin biopsy
b) By imaging, based on the results of an ultrasound
c) Clinically, based on the results of a medical history, family history, and physical examination
d) Based on the results of laboratory tests

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Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Self Test
Severity Assessments
Treatment Question 12
Self-test
Abbreviations Match the Hurley stage with the associated clinical features.
Glossary a) Hurley stage 1 Multiple interconnected fistulas and/or abscesses spread across an entire area of the body (eg, axilla)
References
b) Hurley stage 2 Abscess formation (single or multiple) without fistula or scar formation

c) Hurley stage 3 Recurrent abscesses (single or multiple) with fistula or scar formation; lesions are distinct and widely separated (eg, > 10 cm apart)

Question 13

Which of the following tools might be used to assess HS disease severity? (Select all that apply.)
a) HS-Physician Global Assessment (HS-PGA )
b) Hurley staging system
c) Modified Sartorius score
d) HS Clinical Response

Question 14
Which of the following are risk factors for greater disease severity in patients with HS? (Select all that apply.)

a) No family history of HS
b) Disease duration > 15 years
c) Female sex
d) Male sex
e) Smoking > 15 pack-year history

Question 15
Treatment of HS may achieve all of the following EXCEPT:

a) Clearance or reduction of lesions
b) Getting rid of scars and tunnels beneath the skin
c) Prevention of new lesions
d) A cure

Question 16

Which of the following are treatment options for HS? (Select all that apply.)
a) Gene therapy
b) Cryotherapy
c) Laser surgery
d) Systemic medication therapy

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Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Self Test
Severity Assessments
Treatment Question 17
Self-test
Abbreviations Which of the following factors might affect the treatment strategy for a patient with HS? (Select all that apply.)
Glossary a) Individual lesion type
References b) Disease severity
c) History of acne
d) Potential for adverse events
e) Height
f) Comorbidities

Question 18

Lifestyle modifications for patients with HS may include all of the following EXCEPT:
a) Weight loss
b) Avoidance of tight clothing
c) Phototherapy
d) Smoking cessation

Question 19

Which of the following medications has an indication for the treatment of moderate to severe HS?

a) Systemic antibiotics
b) Adalimumab
c) Topical antibiotics
d) Systemic analgesics

Question 20
Which of the following warnings and precautions are listed in the PI for adalimumab? (Select all that apply.)

a) Serious infections
b) Headache
c) Malignancies
d) Neurologic reactions
e) Rash
f) Hematological reactions

Question 21
Match the type of surgical therapy for HS with its appropriate description.

a) Deroofing Complete excision of an apocrine gland–bearing area of skin

b) Carbon dioxide laser Destroys the hair follicle

c) Conventional Surgery Converts painful recurrent lesions into cosmetically acceptable scars Continued
on Next
d) Nd:YAG laser Vaporizes lesions from inside and out until healthy tissue surrounding lesions is reached Page

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Self Test
Severity Assessments
Treatment Question 22
Self-test
Abbreviations All of the following statements describe the HS-PGA EXCEPT:
Glossary a) Most frequently used assessment tool to measure clinical improvement in clinical trials of medications for HS
References b) Rates lesions on a scale from 0 to 5, from clear to very severe
c) Measures the percentage of BSA affected
d) Assesses the presence and severity of inflammatory nodules, abscesses, and fistulas

Question 23

Which of the following statements describes the HiSCR? (Select all that apply.)
a) Not designed to assess change in disease severity during medication treatment
b) Defined as a ≥ 50% reduction in inflammatory (transient) lesion count (and no increase in abscesses or draining fistulas (chronic inflamed lesions)
compared with baseline
c) A reduction of 50 nodules may be referred to as an AN50 response
d) The 50% reduction threshold for the HiSCR is considered clinically appropriate and meaningful to the patient

Question 24
True/False? A treatment response may be defined as a 50% reduction in HSSI score from baseline to endpoint.

a) True
b) False

Question 25
All of the following statements about the treatment strategy for HS are true EXCEPT:

a) No single treatment will be effective for every patient .
b) Topical therapy is not combined with systemic therapy.
c) Biologic therapy is typically reserved for patients with moderate to severe disease .
d) A multimodal approach that includes lifestyle modifications in addition to medical and/or surgical treatments may be the most effective treatment approach

for most patients with HS

Question 26
Which of the following statements describes the treatment approach recommended by the European S1 guideline ? (Select all that apply.)

a) The treatment approach may be based on the individual subjective impact and objective severity of the disease .
b) Topical medication treatment may be recommended for patients with mild HS.
c) Systemic medications are recommended only for patients with severe HS.
d) Surgical treatment, such as deroofing, laser treatment, or local excision may be recommended for locally recurring lesions, whereas widely spread and/or

more severe lesions may require wide surgical excision.
e) Patients with HS rarely benefit from lifestyle and pain management

Continued
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Page

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Self Test Answers
Severity Assessments
Treatment Question 1
Self-test
Abbreviations HS lesions most commonly appear in which areas of the skin?
Glossary a) Breast, buttocks, pubic area
References b) Chest, scalp, ear
c) Armpit, groin, anal area
d) Arm, leg, back Continued
on Next
Question 2 Page

HS is characterized by which of the following types of skin lesions? (Select all that apply.)
a) Inflammatory nodules
b) Abscesses
c) Scaly plaques
d) Fistulas

Question 3

True/False? HS is characterized by lesions in areas of the skin that have apocrine glands.
a) True
b) False

Question 4
All of the following statements about the epidemiology of HS are true EXCEPT: (Select all that apply.)

a) The prevalence is estimated to be 1% worldwide
b) The typical age of onset is in the early 20s
c) More women are affected than men, in a 3:1 ratio
d) The onset often occurs after menopause

Question 5
Which of the following statements about risk factors for HS are true? (Select all that apply.)

a) 30% to 40% of patients with HS report a family history of HS
b) Obesity quadruples the risk of HS
c) Viral infections increase the risk of HS
d) Cigarette smoking is a well-established risk factor for HS

Question 6

Which of the following statements about the pathophysiology of HS are true? (Select all that apply.)
a) Bacterial infection is the primary cause of HS .
b) The central pathologic event is hyperkeratosis in the hair follicle that leads to plugging and subsequent rupture.
c) HS is caused by pyoderma gangrenosum.
d) The proinflammatory cytokines IL-12, IL-17, IL-23, and TNF are associated with HS lesions.

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Self Test Answers
Severity Assessments
Treatment Question 7
Self-test
Abbreviations All of the following statements accurately describe the disease course of HS EXCEPT:
Glossary a) Patients may have a single, painful bump that persists for weeks or months or may experience breakouts of lesions that clear and reappear.
References b) Nodules that form deep within the dermis may become pus-filled cavities (abscesses) that drain fluid with an unpleasant odor .
c) Severe HS is rarely associated with scarring.
d) Long-standing, poorly controlled HS may be associated with substantial scarring and patients may experience impaired mobility.

Question 8

Which of the following are signs and symptoms of HS? (Select all that apply.)
a) Red, tender bumps that look like boils
b) Painful, pea-sized hard lumps (inflammatory nodules) that develop under the skin
c) Swollen joints
d) Leaking bumps or sores that heal slowly (if at all)

Question 9

True/False? Patients with HS are not significantly more likely than their healthy counterparts to have metabolic syndrome.
a) True
b) False

Question 10

What are the 3 most common comorbidities associated with HS?
a) Pyoderma gangrenosum, spondyloarthritis, and amyloidosis
b) Anemia, amyloidosis, and lymphedema
c) Follicular occlusion syndrome, IBD, spondyloarthritis
d) Plaque psoriasis, IBD, and anemia

Question 11

How is the diagnosis of HS typically made?
a) Histologically, based on the results of a skin biopsy
b) By imaging, based on the results of an ultrasound
c) Clinically, based on the results of a medical history, family history, and physical examination
d) Based on the results of laboratory tests

Continued
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Page

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Self Test Answers
Severity Assessments
Treatment Question 12
Self-test Match the Hurley stage with the associated clinical features.
Abbreviations
Glossary a) Hurley stage 1 Abscess formation (single or multiple) without fistula or scar formation
References
b) Hurley stage 2 Recurrent abscesses (single or multiple) with fistula or scar formation; lesions are distinct and widely separated (eg, > 10 cm apart)

c) Hurley stage 3 Multiple interconnected fistulas and/or abscesses spread across an entire area of the body (eg, axilla)

Question 13 Continued
on Next
Which of the following tools might be used to assess HS disease severity? (Select all that apply.) Page
a) HS-Physician Global Assessment (HS-PGA )
b) Hurley staging system
c) Modified Sartorius score
d) HS Clinical Response

Question 14
Which of the following are risk factors for greater disease severity in patients with HS? (Select all that apply.)

a) No family history of HS
b) Disease duration > 15 years
c) Female sex
d) Male sex
e) Smoking > 15 pack-year history

Question 15
Treatment of HS may achieve all of the following EXCEPT:

a) Clearance or reduction of lesions
b) Getting rid of scars and tunnels beneath the skin
c) Prevention of new lesions
d) A cure
Question 16

Which of the following are treatment options for HS? (Select all that apply.)
a) Gene therapy
b) Cryotherapy
c) Laser surgery
d) Systemic medication therapy

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Self Test Answers
Severity Assessments
Treatment Question 17
Self-test
Abbreviations Which of the following factors might affect the treatment strategy for a patient with HS? (Select all that apply.)
Glossary a) Individual lesion type
References b) Disease severity
c) History of acne
d) Potential for adverse events
e) Height
f) Comorbidities

Question 18

Lifestyle modifications for patients with HS may include all of the following EXCEPT:
a) Weight loss
b) Avoidance of tight clothing
c) Phototherapy
d) Smoking cessation

Question 19

Which of the following medications has an indication for the treatment of moderate to severe HS?

a) Systemic antibiotics
b) Adalimumab
c) Topical antibiotics
d) Systemic analgesics

Question 20

Which of the following warnings and precautions are listed in the PI for adalimumab? (Select all that apply.)
a) Serious infections
b) Headache
c) Malignancies
d) Neurologic reactions
e) Rash
f) Hematological reactions

Question 21

Match the type of surgical therapy for HS with its appropriate description.

a) Deroofing Converts painful recurrent lesions into cosmetically acceptable scars

b) Carbon dioxide laser Vaporizes lesions from inside and out until healthy tissue surrounding lesions is reached

c) Conventional Surgery Complete excision of an apocrine gland–bearing area of skin Continued
on Next
d) Nd:YAG laser Destroys the hair follicle Page

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Self Test Answers
Severity Assessments
Treatment Question 22
Self-test
Abbreviations All of the following statements describe the HS-PGA EXCEPT:
Glossary a) Most frequently used assessment tool to measure clinical improvement in clinical trials of medications for HS
References b) Rates lesions on a scale from 0 to 5, from clear to very severe
c) Measures the percentage of BSA affected
d) Assesses the presence and severity of inflammatory nodules, abscesses, and fistulas

Question 23

Which of the following statements describes the HiSCR? (Select all that apply.)
a) Not designed to assess change in disease severity during medication treatment
b) Defined as a ≥ 50% reduction in inflammatory (transient) lesion count (and no increase in abscesses or draining fistulas (chronic inflamed lesions)
compared with baseline
c) A reduction of 50 nodules may be referred to as an AN50 response
d) The 50% reduction threshold for the HiSCR is considered clinically appropriate and meaningful to the patient

Question 24
True/False? A treatment response may be defined as a 50% reduction in HSSI score from baseline to endpoint.

a) True
b) False

Question 25
All of the following statements about the treatment strategy for HS are true EXCEPT:

a) No single treatment will be effective for every patient .
b) Topical therapy is not combined with systemic therapy.
c) Biologic therapy is typically reserved for patients with moderate to severe disease .
d) A multimodal approach that includes lifestyle modifications in addition to medical and/or surgical treatments may be the most effective treatment approach

for most patients with HS

Question 26
Which of the following statements describes the treatment approach recommended by the European S1 guideline ? (Select all that apply.)

a) The treatment approach may be based on the individual subjective impact and objective severity of the disease.
b) Topical medication treatment may be recommended for patients with mild HS.
c) Systemic medications are recommended only for patients with severe HS.
d) Surgical treatment, such as deroofing, laser treatment, or local excision may be recommended for locally recurring lesions, whereas widely spread and/or

more severe lesions may require wide surgical excision.
e) Patients with HS rarely benefit from lifestyle and pain management

This material is for your information and/or training only. It cannot be shown to or otherwise used with members of the Healthcare Community.
Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Abbreviations
Severity Assessments
Treatment BMI = body mass index
Self-test C = C-reactive protein
Abbreviations DLQI = Dermatology Life Quality Index
Glossary EADV = European Academy of Dermatology and Venereology
References ESR = erythrocyte sedimentation rate
FDA = Food and Drug Administration
HDL = high-density lipoprotein
HiSCR = Hidradenitis Suppurativa Clinical Response
HS = hidradenitis suppurativa
HRQoL = health-related quality of life
HS-PGA = Hidradenitis Suppurativa-Physician Global Assessment
HSSI = Hidradenitis Suppurativa Severity Index
IBD = inflammatory bowel disease
IL = interleukin
Nd:YAG = neodymium-doped yttrium aluminum garnet
PI = prescribing information
PRO = patient-reported outcome
TNF = tumor necrosis factor
WBC = white blood cell

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Glossary
Severity Assessments
Treatment Abscess: a cavity formed within solid tissue that is filled with purulent exudate of health-related quality of life based on a 10-item scale that measures 6
Self-test and is associated with redness, pain, and swelling6 domains (symptoms and feelings, daily activities, leisure, work or school,
Abbreviations Acne inversa: previously used name for hidradenitis suppurativa1 personal relationships, and treatment side effects)35
Glossary Amyloidosis: a disease characterized by the accumulation of amyloid protein Dermis: layer of skin that contains blood and lymphatic vessels, nerves and
References outside the cells of various tissues and organs in the body6 nerve endings, glands, and, except on hairless areas of the skin, hair follicles6
Analgesic: a medication that relieves pain17 Epidermal: relating to the outermost layer of the skin6
Anemia: reduction in mass of circulating red blood cells17 Epithelium: layer of cells that forms the epidermis of the skin and the surface
Apocrine gland: gland that opens into hair follicles and releases its layer of mucosal membranes; lacks blood vessels17
cytoplasmic contents (sweat or milk)17 Erythema: redness due to dilation of small blood vessels; usually associated
Autosomal dominant: a dominant gene found on any chromosome other with inflammation6
than the X or Y chromosome17; a dominant gene is the gene that is expressed6 Erythrocyte sedimentation rate (ESR): rate of settling of red blood cells in
Biologic: medicine derived from human genes that is man-made through anticoagulated blood; rate increases in inflammatory states6
genetic engineering techniques and closely related to a protein that occurs Fistula: an abnormal passage from one epithelial surface to another epithelial
naturally in the body33 surface6
Biosimilar: biological product that is “highly similar” to an approved biologic Follicular occlusion syndrome: disorders that involve occlusion of follicles,
(known as a reference product) already being used to treat patients25; to be including acne conglobata, dissecting cellulitis of the scalp, HS, and pilonidal
classified as a biosimilar, the protein must have no clinically meaningful cysts15
differences in terms of safety and effectiveness from the reference product; Hair follicle: an indentation in the epidermis that forms a cylindrical
only minor differences in clinically inactive components are allowed25 depression that holds the hair root17
Body surface area (BSA): area of the external surface of the body (ie, skin) Health-related Quality of Life (HRQoL): impact of health status on quality of
typically expressed in square meters (m2)6 life; a multidimensional concept that includes aspects of physical, mental,
Combination therapy: using one or more therapies with different mechanisms emotional, and social functioning; a patient-reported outcome36
of action in combination for treatment; using one or more types of medications, Hidradenitis suppurativa (HS): chronic inflammatory skin disease
such as topical and systemic; for HS, this may also refer to use of medication characterized by swollen, painful lesions occurring in the sweat-gland-bearing
treatment and surgical treatment1 skin of the armpit, groin, anal, and breast regions; pus-filled abscesses may
C-reactive protein (CRP): a beta-globulin found in elevated levels in the progress to hard lumps that may progress further to deep-seated inflamed
serum of various people with certain inflammatory diseases6 lesions with chronic seepage, and healing of affected areas is typically
Crohn’s disease: chronic inflammatory bowel disease that can affect any part associated with progressive scarring8
of the gastrointestinal (GI) tract as well as the entire thickness of the bowel Hyperkeratosis: increased production of keratinocytes within the hair follicle;
wall; associated with abdominal discomfort and frequent emptying of the thickening of the epidermis6
colon34 Induration: a region of firm, hardened tissue6
Cyst: an abnormal sac with a membranous lining that contains gas, fluid, or a Inflammatory bowel disease: general term for Crohn’s disease and ulcerative
semisolid material6 colitis, chronic disorders of the small and large intestine, of unknown cause,
Cytokine: any of several proteins produced by many cell types that regulates with conspicuous inflammatory features and distinctive but overlapping signs
the intensity and duration of immune responses and mediates cell-to-cell and symptoms6
communication6 Inguinal: relating to the groin area; junction between the legs and the pelvic
Dendritic cell: also called Langerhans cell in skin; antigen-presenting cell area6
capable of activating naïve T cells and important for initiation of adaptive
immune responses to protein antigens6 Continued
Dermatology Life Quality Index (DLQI): a patient-reported outcome measure on Next
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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis Glossary Spondyloarthritis: a group of rheumatic inflammatory diseases that share
Severity Assessments clinical and genetic features, including involvement of the axial skeleton,
Treatment Interleukin: name given to a group of multifunctional cytokines after their peripheral arthritis, enthesitis, dactylitis, uveitis, human leukocytic antigen B27
Self-test amino acid structure is known; synthesized by lymphocytes, monocytes, (HLA-B27) positivity, and comorbid psoriasis and/or IBD37
Abbreviations macrophages, and other cells6; important interleukins in HS include IL-12, IL- Subcutaneous: beneath the skin6
Glossary 17, and IL-231 T helper cell: subset of T cells that secretes various cytokines that regulate
References Intertriginous: occurring between folds or opposing surfaces of skin; for the immune response6; T cells are immune cell that matures in the thymus and
example, between the pelvic region and thigh, or in the armpit6 are responsible for cell-mediated immunity6
Keratin: a fibrous structural protein6 Th17: subset of T cells that release proinflammatory cytokines, for example, IL-
Keratinocyte: cell in the epidermis that produces keratin (a fibrous structural 17A, IL-17F, and IL-2210
protein) and can also secrete cytokines (eg, IL-10, IL-20)6 Tumor necrosis factor (TNF): a key proinflammatory cytokine produced by
Lymphedema: swelling of lymphatic vessels in subcutaneous tissues and macrophages and many other cells that regulates the immune system17
accumulation of large amounts of lymph in the affected area6 Ultrasound: the use of high frequency sound waves to obtain images for
Maceration: softening of tissue by the action of liquid (such as sweat)6 medical diagnostic purposes6
Macrophage: immune cell that phagocytizes foreign substances and is Venereology: the study of sexually transmitted diseases17
involved in initiating and regulating immune responses6 White blood cell (WBC): any of various blood cells that help protect the body
Metabolic syndrome: a cluster of conditions that is associated with increased from infection and disease; also called leukocytes6
risk of cardiovascular disease6
Monocyte: most common type of white blood cell; plays primary role in
inflammation; is readily attracted to foreign antigens and destroys them by
phagocytosis17
Monotherapy: using a single type of therapy, such as a medication therapy,1
or a single type of medication therapy17
Neutrophilic: describing neutrophils, which play primary role in inflammation;
neutrophils are readily attracted to foreign antigens and destroy them by
phagocytosis17 (the process of ingestion and digestion by cells of solid
substances such as other cells, bacteria, bits of necrotic tissue, foreign
particles6
Nodule: a well-defined, discrete knot in the skin up to 1.0 cm in diameter that
is solid with palpable depth6
Patient-related outcome (PRO): an assessment of a patient’s health
condition from the patient’s point of view17
Plaque: a well-defined, raised, thickened, solid area of the skin that exceeds
1 cm in diameter6
Plaque psoriasis: common chronic inflammatory disease of the skin
characterized by the development of raised red, scaly patches on the skin6
Pyoderma gangrenosum: an uncommon inflammatory skin disease that
involves formation of large, painful ulcers (often on the legs) that are
associated with an infiltration of neutrophils2,17

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.

Background Hidradenitis Suppurativa (HS) – Disease State
Pathophysiology
Diagnosis References
Severity Assessments
Treatment 1. Zouboulis CC, Desai N, Emtestam L, et al. European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa. J Eur Acad Dermatol
Self-test
Abbreviations Venereol. 2015;29:619-644.
Glossary
References 2. Dufour DN, Emtestam L, Jemec GB. Hidradenitis suppurativa: a common and burdensome, yet under-recognised, inflammatory skin disease.

Postgrad Med J. 2014;90:216-221.

3. Woodruff CM, Charlie AM, Leslie KS. Hidradenitis suppurativa: a guide for the practicing physician. Mayo Clin Proc. 2015;90:1679-1693.

4. Access Emergency Medicine. Disorders of Sebaceous and Apocrine Glands. http://accessemergencymedicine.mhmedical.com/content.

aspx?bookid=349&sectionid=40453098. Accessed July 31, 2017.

5. American Academy of Dermatology. Hidradenitis suppurativa. 2015. https://www.aad.org/public/diseases/painful-skin-joints/hidradenitis-

suppurativa. Accessed June 7, 2016.

6. Stedman’s Online Medical Dictionary. 2016. http://www.stedmansonline.com. Accessed June 24, 2016.

7. Jemec GB. Clinical practice: hidradenitis suppurativa. N Engl J Med. 2012;366:158-164.

8. National Organization for Rare Disorders. Hidradenitis suppurativa. 2012. http://rarediseases.org/rare-diseases/hidradenitis-suppurativa/.

Accessed June 24, 2016.

9. Kimball AB, Kerdel F, Adams D, et al. Adalimumab for the treatment of moderate to severe hidradenitis suppurativa: a parallel randomized trial.

Ann Intern Med. 2012;157:846-855.

10. Boehncke WH and Schön MP. Psoriasis. Lancet. 2015;386:983-994.

11. Revuz JE, Canoui-Poitrine F, Wolkenstein P, et al. Prevalence and factors associated with hidradenitis suppurativa: results from two case-control

series. J Am Acad Dermatol. 2008;59:596-601.

12. Prens E and Deckers I. Pathophysiology of hidradenitis suppurativa: an update. J Am Acad Dermatol. 2015;73(5)(suppl 1):S8-S11.

13. Mayo Clinic. Hidradenitis suppurativa: symptoms and causes. 2016. http://www.mayoclinic.org/diseases-conditions/hidradenitis-

suppurativa/symptoms-causes/dxc-20200019. Accessed June 15, 2016.

14. Alikhan A. Hidradenitis Suppurativa. JAMA Dermatol. 2016; 152:736.

15. Fimmel S and Zouboulis CC. Comorbidities of hidradenitis suppurativa (acne inversa). Dermatoendocrinol. 2010;2:9-16.

16. Menter A, Griffiths CE, Tebbey PW, Horn EJ, Sterry W; on behalf of the International Psoriasis Council. Exploring the association between

cardiovascular and other disease-related risk factors in the psoriasis population: the need for increased understanding across the medical

community. J Eur Acad Dermatol Venereol. 2010;24:1371-1377.

17. Taber’s Online Medical Dictionary. 2016. http://www.tabers.com/tabersonline/. Accessed June 24, 2016.

18. van der Zee HH and Jemec GB. New insights into the diagnosis of hidradenitis suppurativa: clinical presentations and phenotypes. J Am Acad

Dermatol. 2015;73(suppl 1):S23-S26.

19. Faivre C, Villani AP, Aubin F, et al. Hidradenitis suppurativa (HS): An unrecognized paradoxical effect of biologic agents (BA) used in chronic

inflammatory diseases. J Am Acad Dermatol. 2016;74:1153-1159.

20. Kimball AB, Sobell JM, Zouboulis CC, et al. HiSCR (Hidradenitis Suppurativa Clinical Response): a novel clinical endpoint to evaluate therapeutic

outcomes in patients with hidradenitis suppurativa from the placebo-controlled portion of a phase 2 adalimumab study. J Eur Acad Dermatol

Venereol. 2016;30:989-994.

21. Hidradenitis Suppurativa Foundation, Inc. Treatment for hidradenitis suppurativa. 2014. http://www.hs-foundation.org/about-hs/treatment/. Continued
Accessed June 24, 2016. on Next

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Any links to external websites and the articles or content contained therein are included as part of this guidance. © 2017 Amgen Inc. All rights reserved.


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