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Hidradenitis suppurativa

Authors: Vanessa Ngan, Staff Writer, Dr Amanda Oakley, Dermatologist, Hamilton, New Zealand. 1997. Updated December 2015. Revised by: Dr Abdulhadi Jfri, PGY-5 Dermatology Resident, Division of Dermatology, McGill University, Montreal, Quebec, Canada. Dr Elizabeth O’Brien, Assistant Professor of Dermatology, Division of Dermatology, McGill University, Montreal, Quebec, Canada. Copy edited by Gus Mitchell. January 2021.


Hidradenitis suppurativa — codes and concepts
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What is hidradenitis suppurativa?

Hidradenitis suppurativa (HS), also called acne inversa, is a chronic inflammatory skin condition that affects apocrine gland-bearing skin in the axillae, groin, and under the breasts. It is characterised by persistent or recurrent boil-like nodules and abscesses that culminate in a purulent discharge, sinuses, and scarring.

HS can have a significant psychological impact, and many patients suffer from anxiety, depression, and impairment of body image.

Hidradenitis suppurativa of the axilla

Who gets hidradenitis suppurativa?

Hidradenitis suppurativa often starts at puberty, is most active between the ages of 20 and 40 years, and in women can resolve at menopause. It is three times more common in females than in males. Associations and risk factors include:

What causes hidradenitis suppurativa?

Although ‘hidradenitis’ implies an inflammatory disease of the sweat glands, we now know that HS is an autoinflammatory syndrome. The exact pathogenesis is not yet understood. Factors involved in the development of acne inversa include:

  • Follicular occlusion
  • An abnormal cutaneous or follicular microbiome
  • Release of pro-inflammatory cytokines
  • Inflammation causing rupture of the follicular wall, destroying sebaceous and apocrine glands and ducts.

What are the clinical features of hidradenitis suppurativa?

Acne inversa can affect single or multiple areas in the axillae, neck, inframammary fold, and inner upper thighs. Anogenital involvement most commonly affects the groin, mons pubis, vulva, scrotum, perineum, buttocks, and perianal folds.

HS is characterised clinically by:

  • Open double-headed comedones
  • Painful firm papules and nodules
  • Pustules, fluctuant pseudocysts, and abscesses
  • Draining sinuses linking inflammatory lesions
  • Hypertrophic and atrophic scars.

Characteristic lesions of hidradenitis suppurativa

Clinical phenotypes of hidradenitis suppurativa (HS)

Phenotype Clinical features
Regular HS
  • Most common form
  • Recurrent inflammatory lesions in typical locations for at least 6 months
Frictional furuncle
  • Regular HS plus multiple deep nodules and abscesses on sites exposed to enhanced friction (abdomen, thighs, buttocks)
Scarring folliculitis
  • Regular HS plus pustules, cysts, superficial nodules, depressed cribriform scarring, and double-headed comedones
  • Significant scarring
Conglobata
  • Cyst formation and acne conglobata lesions on the back and face
Syndromic
  • HS in conjunction with syndromic features (PAPA, PASH, PAPASH)

Clinical phenotypes of hidradenitis suppurativa

How is the severity of hidradenitis suppurativa assessed?

Disease severity and extent is measured by clinical and ultrasound assessment at the time of diagnosis and when monitoring response to treatment. There are a number of severity scales for HS [see guidelines for Hidradenitis suppurativa: severity assessment].

The Hurley system, the most widely used assessment tool, describes three clinical stages.

  • Stage I: solitary or multiple isolated abscess formation without sinus tracts or scarring.
  • Stage II: Recurrent abscesses, single or multiple widely spaced lesions, with sinus tract formation.
  • Stage III: Diffuse involvement of an area with multiple interconnected sinus tracts and abscesses.

Hidradenitis suppurativa in skin of colour

What are the complications of hidradenitis suppurativa?

Complications of HS can include:

How is hidradenitis suppurativa diagnosed?

The diagnosis of acne inversa requires all three components of the triad to be met:

  • Characteristic lesions
  • Typical distribution
  • Presence and recurrence of lesions.

Swabs for bacteriology are typically negative, which is a clue to diagnosis. Extensive investigations are rarely required. Investigations may be indicated to exclude a differential diagnosis, for possible complications, to identify comorbidities, or for planned treatment.

What is the differential diagnosis for hidradenitis suppurativa?

Differential diagnoses for hidradenitis suppurativa can include the following conditions.

What is the treatment for hidradenitis suppurativa?

For a detailed evidence-based review of treatment for HS, see Management of hidradenitis suppurativa: an Australasian consensus statement. The following provides important general measures and additional medical treatments.

General measures

General measures for treating patients with hidradenitis suppurativa include:

  • Weight loss
  • Smoking cessation
  • Loose fitting clothing
  • Absorbent dressings
  • Analgesics
  • Management of anxiety and depression; including reassurance that the condition is not infectious or a results of poor hygiene.

Specific medical measures

Specific medical measures for treating hidradenitis suppurativa include:

Topical treatments

Systemic treatments

Antibiotics for bacterial infections

  • Short oral course for acute staphylococcal abscess
  • Tetracyclines as a single agent
  • Prolonged courses of at least three months of combination antibiotics: clindamycin plus rifampicin; tetracyclines plus rifampicin; fluoroquinolone plus metronidazole plus rifampicin
  • Intravenous ertapenem.

Other oral treatments

Surgical and other procedural measures

  • Incision and drainage of acute abscesses
  • Local excision of persistent nodules, abscesses, and sinuses
  • Deroofing and curettage of persistent abscesses and sinuses
  • Radical excisional surgery of an entire affected area
  • Laser ablation (CO2) of nodules, abscesses, and sinuses
  • Laser/light hair removal.

What is the outcome for hidradenitis suppurativa?

Hidradenitis suppurativa tends to improve in pregnancy in those who usually have flares during menstruation. Normal vaginal delivery is possible unless the patient has extensive painful genital lesions.

HS is a chronic scarring condition. Spontaneous remission may occur with time, but scarring persists.

Early diagnosis and treatment are required to minimise lasting damage.

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Bibliography

  • 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(6):1153–9. doi:10.1016/j.jaad.2016.01.018. PubMed
  • Goldburg SR, Strober BE, Payette MJ. Hidradenitis suppurativa: epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol. 2020;82(5):1045–58. doi:10.1016/j.jaad.2019.08.090. PubMed
  • Goldburg SR, Strober BE, Payette MJ. Hidradenitis suppurativa: current and emerging treatments. J Am Acad Dermatol. 2020;82(5):1061–82. doi:10.1016/j.jaad.2019.08.089. PubMed
  • Kohorst JJ, Baum CL, Otley CC, et al. Surgical management of hidradenitis suppurativa: outcomes of 590 consecutive patients. Dermatol Surg. 2016;42(9):1030–40. doi:10.1097/DSS.0000000000000806. PubMed
  • Lee JH, Kwon HS, Jung HM, Kim GM, Bae JM. Prevalence and comorbidities associated with hidradenitis suppurativa in Korea: a nationwide population-based study. J Eur Acad Dermatol Venereol. 2018;32(10):1784–90. doi:10.1111/jdv.15071. PubMed
  • Lim SYD, Oon HH. Systematic review of immunomodulatory therapies for hidradenitis suppurativa. Biologics. 2019;13:53–78. doi:10.2147/BTT.S199862. PubMed
  • Ring HC, Thorsen J, Saunte DM, et al. The follicular skin microbiome in patients with hidradenitis suppurativa and healthy controls. JAMA Dermatol. 2017;153(9):897–905. doi:10.1001/jamadermatol.2017.0904. PubMed
  • Schrader AM, Deckers IE, van der Zee HH, Boer J, Prens EP. Hidradenitis suppurativa: a retrospective study of 846 Dutch patients to identify factors associated with disease severity. J Am Acad Dermatol. 2014;71(3):460–7. doi:10.1016/j.jaad.2014.04.001. PubMed
  • van der Zee HH, Boer J, Prens EP, Jemec GB. The effect of combined treatment with oral clindamycin and oral rifampicin in patients with hidradenitis suppurativa. Dermatology. 2009;219(2):143–7. doi:10.1159/000228337. PubMed
  • Vossen AR, van Straalen KR, Prens EP, van der Zee HH. Menses and pregnancy affect symptoms in hidradenitis suppurativa: a cross-sectional study. J Am Acad Dermatol. 2017;76(1):155–6. doi:10.1016/j.jaad.2016.07.024. PubMed

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