Continuing medical education
Hidradenitis suppurativa: Epidemiology, clinical presentation, and pathogenesis

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Hidradenitis suppurativa (HS) is an inflammatory disorder that is characterized by chronic deep-seated nodules, abscesses, fistulae, sinus tracts, and scars in the axilla, inguinal area, submammary folds, and perianal area. This disfiguring condition is accompanied by pain, embarrassment, and a significantly decreased quality of life. Although the mechanism of HS has not been entirely elucidated, lesion formation is believed to center around follicular hyperkeratosis within the pilosebaceous–apocrine unit. Recent research has provided new insight into the role of cytokines in the pathogenesis of HS, helping close some existing knowledge gaps in the development of this condition. The first article in this continuing medical education series reviews HS epidemiology, clinical presentation, and classification. We also provide an update on the most recent understanding of HS pathogenesis, including the central role of inflammatory cytokines and other contributing factors, such as genetics, hormones, and pathogenic microorganisms.

Section snippets

Epidemiology

Key points

  1. The prevalence of HS is unknown, but estimates range from 0.00033% to 4.10%

  2. HS most frequently occurs in young adults

  3. HS is more than twice as common in women compared with men, and is more common in African Americans and biracial individuals than whites

HS prevalence is unclear. Estimates range from 0.00033% to 4.10%.5 Higher estimates are derived from prospective6 and self-reported studies7,8; lower estimates derive from registries.9, 10, 11, 12 A retrospective analysis of >48 million patients

Quality of life

Key points

  1. HS significantly decreases patients' quality of life

  2. Depression and pain are associated with HS

  3. HS leads to social and work impairment

HS dramatically decreases quality of life (QoL). Patients report embarrassment, self-consciousness, and an inability to participate in social and athletic activities.17 Depression, anxiety, pain, high body mass index (BMI), and work impairment are the main factors affecting QoL.18,19 Depression occurs in 5.9% to 42.9% of patients with HS15,19, 20, 21, 22 in both

Presentation and clinical classification

Key points

  1. HS is diagnosed by clinical features and history, and there are multiple scoring systems used for the classification of disease severity

  2. Lesions include deep-seated nodules, abscesses, and sinus tracts that rupture and form scars

  3. The Hurley staging system is the most widely used HS classification system

  4. There are several clinically distinct HS phenotypes

HS is diagnosed clinically; pathologic confirmation is unnecessary.31, 32, 33 There are 3 criteria for diagnosis: characteristic lesions,

Comorbidities and risk factors

Key points

  1. HS is associated with many comorbidities, most of which are inflammatory in nature

  2. Obesity is the most common comorbidity associated with HS

  3. There is a strong association between HS and tobacco smoking

  4. There is an increased prevalence of HS among psoriasis patients

  5. HS is a component of the follicular occlusion triad/tetrad

HS patients have an incredibly high comorbidity burden.57,58 Hypertension, obesity, dyslipidemia, thyroid disorder, arthropathies, psychiatric disorders, and polycystic ovarian

Pathogenesis

Key points

  1. The primary event in HS is follicular hyperkeratosis, leading to rupture of the hair follicle and subsequent inflammation of apocrine glands

  2. TNF-α and interleukin-17 are key cytokines in HS pathogenesis

  3. Levels of TNF-α are higher in the lesional tissue of patients with HS than that of patients with psoriasis

  4. Levels of TNF-α in lesional HS skin and serum levels of interleukin-17 correlate with disease severity

  5. The role of sex hormones in HS remains unclear

  6. Thirty percent to 40% of patients with HS

Bacterial colonization

Key points

  1. HS patients have a unique skin microbiome

  2. Biofilms are a key feature of lesional skin, but distinct species have also been found

  3. There is a lack of consensus on which bacterial species are most common in HS lesions

The cutaneous microbiome is significantly different in HS lesional skin, nonlesional skin, and patients without HS.81 The lesional skin microbiome consists predominantly of Corynebacterium, Porphyromonas, and Peptoniphilus species,129 while nonlesional skin has predominantly

References (140)

  • B.B. Dhaou et al.

    Hidradenitis suppurativa (Verneuil's disease)

    J Saudi Soc Dermatol Dermatol Surg

    (2013)
  • A. Grant et al.

    Infliximab therapy for patients with moderate to severe hidradenitis suppurativa: a randomized, double-blind, placebo-controlled crossover trial

    J Am Acad Dermatol

    (2010)
  • H.H. van der Zee et al.

    New insights into the diagnosis of hidradenitis suppurativa: clinical presentations and phenotypes

    J Am Acad Dermatol

    (2015)
  • J. Shlyankevich et al.

    Hidradenitis suppurativa is a systemic disease with substantial comorbidity burden: a chart-verified case-control analysis

    J Am Acad Dermatol

    (2014)
  • T. Ergun

    Hidradenitis suppurativa and the metabolic syndrome

    Clin Dermatol

    (2018)
  • I.M. Miller et al.

    Prevalence, risk factors, and comorbidities of hidradenitis suppurativa

    Dermatol Clin

    (2016)
  • B. Moran et al.

    Hidradenitis suppurativa is characterized by dysregulation of the Th17:Treg cell axis, which is corrected by anti-TNF therapy

    J Invest Dermatol

    (2017)
  • D. Jiménez-Gallo et al.

    Effects of adalimumab on T-helper-17 lymphocyte- and neutrophil-related inflammatory serum markers in patients with moderate-to-severe hidradenitis suppurativa

    Cytokine

    (2018)
  • W. Chen et al.

    Should hidradenitis suppurativa/acne inversa best be renamed as “dissecting terminal hair folliculitis”?

    Exp Dermatol

    (2017)
  • E. Shavit et al.

    Proceeding report of the Second Symposium on Hidradenitis Suppurativa Advances (SHSA) 2017

    Exp Dermatol

    (2019)
  • J.R. Ingram

    Hidradenitis suppurativa: an update

    Clin Med

    (2016)
  • J.M. Von Der Werth et al.

    The clinical genetics of hidradenitis suppurativa revisited

    Br J Dermatol

    (2000)
  • C.J. Posso-De Los Rios et al.

    Proceeding report of the third symposium on Hidradenitis Suppurativa advances (SHSA) 2018

    Exp Dermatol

    (2019)
  • G.R. Vinding et al.

    The prevalence of inverse recurrent suppuration: a population-based study of possible hidradenitis suppurativa

    Br J Dermatol

    (2014)
  • G.B. Jemec

    The symptomatology of hidradenitis suppurativa in women

    Br J Dermatol

    (1988)
  • A. Mahé et al.

    Skin diseases in Bamako (Mali)

    Int J Dermatol

    (1998)
  • A. Garg et al.

    Sex- and age-adjusted population analysis of prevalence estimates for hidradenitis suppurativa in the United States

    JAMA Dermatol

    (2017)
  • M. Slyper et al.

    Incidence of sexual dysfunction among patients with hidradenitis suppurativa: a population-based retrospective analysis

    Br J Dermatol

    (2018)
  • E. Delany et al.

    A cross-sectional epidemiological study of hidradenitis suppurativa in an Irish population (SHIP)

    J Eur Acad Dermatol Venereol

    (2018)
  • R. Vangipuram et al.

    Factors contributing to depression and chronic pain in patients with hidradenitis suppurativa: results from a single-center retrospective review

    Dermatology

    (2016)
  • E. Shavit et al.

    Psychiatric comorbidities in 3207 patients with hidradenitis suppurativa

    J Eur Acad Dermatol Venereol

    (2015)
  • Ł. Matusiak

    Profound consequences of hidradenitis suppurativa: a review

    Br J Dermatol

    (2019)
  • Ł. Matusiak et al.

    Clinical characteristics of pruritus and pain in patients with hidradenitis suppurativa

    Acta Derm Venereol

    (2018)
  • Z.S. Patel et al.

    Pain, psychological comorbidities, disability, and impaired quality of life in hidradenitis suppurativa [corrected]

    Curr Pain Headache Rep

    (2017)
  • A. Kouris et al.

    Quality of life and psychosocial implications in patients with hidradenitis suppurativa

    Dermatology

    (2016)
  • A.J. Onderdijk et al.

    Depression in patients with hidradenitis suppurativa

    J Eur Acad Dermatol Venereol

    (2013)
  • R. Kjaersgaard Andersen et al.

    Factors predicting the self-evaluated health of hidradenitis suppurativa patients recruited from an outpatient clinic

    J Eur Acad Dermatol Venereol

    (2018)
  • J. Gasparic et al.

    Recognizing syndromic hidradenitis suppurativa: a review of the literature

    J Eur Acad Dermatol Venereol

    (2017)
  • R.G. Micheletti

    Natural history, presentation, and diagnosis of hidradenitis suppurativa

    Semin Cutan Med Surg

    (2014)
  • G. Shalom

    Hidradenitis suppurativa: epidemiology, clinical features, associated comorbidities and treatment

    G Ital Dermatol Venereol

    (2017)
  • D.A. Vekic et al.

    Hidradenitis suppurativa, a review of pathogenesis, associations and management. Part 1

    Australas J Dermatol

    (2018)
  • S. Hessam et al.

    A novel severity assessment scoring system for hidradenitis suppurativa

    JAMA Dermatol

    (2018)
  • I. Parulkar et al.

    Epidemiologic and clinical features of hidradenitis suppurativa

    Semin Cutan Med Surg

    (2017)
  • W.B. Kim et al.

    Clinical features and patient outcomes of hidradenitis suppurativa: a cross-sectional retrospective study

    J Cutan Med Surg

    (2016)
  • C.C. Zouboulis et al.

    Hidradenitis suppurativa/acne inversa: criteria for diagnosis, severity assessment, classification and disease evaluation

    Dermatology

    (2015)
  • D.M. Saunte et al.

    Diagnostic delay in hidradenitis suppurativa is a global problem

    Br J Dermatol

    (2015)
  • M.K. Smith et al.

    Hidradenitis suppurativa: an update on connecting the tracts

    F1000Res

    (2017)
  • H.J. Hurley

    Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa and familial benign pemphigus. Surgical approach

  • L. Thorlacius et al.

    Inter-rater agreement and reliability of outcome measurement instruments and staging systems used in hidradenitis suppurativa

    Br J Dermatol

    (2019)
  • K. Sartorius et al.

    Suggestions for uniform outcome variables when reporting treatment effects in hidradenitis suppurativa

    Br J Dermatol

    (2003)
  • Cited by (0)

    Funding sources: None.

    Dr Strober serves as consultant for AbbVie, Almirall, Amgen, Arena, Aristea, Boehringer Ingelheim, Bristol-Myers-Squibb, Celgene, Dermavant, Dermira, Janssen, Leo, Eli Lilly, Kyowa Hakko Kirin, Meiji Seika Pharma, Novartis, Pfizer, GlaxoSmithKline, UCB Pharma, Sun Pharma, Ortho Dermatologics, Regeneron, Sanofi-Genzyme. Dr Strober is also a speaker for AbbVie, Lilly, Janssen, Ortho Dermatologics. Dr Strober is also a scientific director for Corrona Psoriasis Registry. Dr Strober is also an investigator for Dermavant, AbbVie, Corrona Psoriasis Registry, Dermira. Dr Payette is a consultant for Abbvie Inc, Novartis, and Janssen Global Services.

    Date of release: May 2020

    Expiration date: May 2023

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