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Hidradenitis Suppurativa
Gregor B.E. Jemec, M.D., D.M.Sc.
This Journal feature begins with a case vignette highlighting a common clinical problem. Evidence supporting various strategies is then presented, followed by a review of formal guidelines, when they exist. The article ends with the author’s clinicalrecommendations.

A 36-year-old woman has recurrent boils under both arms and in the groin. They flare premenstrually, causing pain, suppuration, and an offensive odor. Scarring has developed in the groin area, and chronically draining sinus tracts are interspersed with normal skin. Treatment with short courses of antibiotics or with incision and drainage has had no apparent effect, and she hasbecome socially isolated because of embarrassment about her condition. How would you manage this case?

The Cl inic a l Probl em
From the Department of Dermatology, Roskilde Hospital, Roskilde, Denmark; and Faculty of Health Sciences, University of Copenhagen, Copenhagen. Address reprint requests to Dr. Jemec at the Department of Dermatology, Roskilde Hospital, Roskilde DK-4000, Denmark, or atgbj@regionsjaelland.dk. N Engl J Med 2012;366:158-64.
Copyright © 2012 Massachusetts Medical Society.

An audio version of this article is available at NEJM.org

Hidradenitis suppurativa1,2 is a chronic, recurrent inflammatory disease affecting skin that bears apocrine glands. It usually develops after puberty, manifested as painful, deep-seated, inflamed lesions, including nodules, sinustracts, and abscesses. In most patients, flares are accompanied by increased pain and suppuration at varying intervals, often occurring premenstrually in women. If untreated, the flares subside within 7 to 10 days.3 European studies have suggested that hidradenitis suppurativa is not a rare disease. A French community-based study,4 in which persons older than 15 years of age responded to a validatedquestionnaire (with a positive predictive value of 85 to 89%), showed a point prevalence at 1 year of 1%. Studies of young adults (18 to 33 years of age) undergoing screening for sexually transmitted diseases have shown point prevalences of up to 4%.5 Women are more frequently affected than men (female:male ratio, 3:1) and appear to be more likely to have genitofemoral lesions. The condition mostcommonly develops in persons in their early 20s, although the onset has been described in prepubertal children and in postmenopausal women as well.4,6 The prevalence of the disease appears to decline at an age of more than 50 years.4 About one third of patients with hidradenitis suppurativa report a family history of the disease, and affected families with an autosomal dominant mode of inheritancehave been identified. In a small number of cases in which hidradenitis suppurativa is accompanied by severe acne and perifolliculitis capitis, the disease has been linked to chromosome 1p21.1–1q25.3 and mutations of the γ-secretase complex.7 Studies have not shown associations between HLA antigens and hidradenitis suppurativa.8 Cigarette smoking is a recognized risk factor for both the developmentof hidradenitis suppurativa and severe disease.9 Obesity is also a risk factor; the majority of patients are overweight, and both body-mass index and tobacco smoking have been directly correlated with the severity of this condition.10 The disease has a substantial negative effect on the quality of life of affected persons, as compared with the general population or with patients who have otherchronic skin conditions (e.g., psoriasis or eczematous dermatitis).11-13 Rates of sick

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The New England Journal of Medicine Downloaded from nejm.org on September 16, 2012. For personal use only. No other uses without permission. Copyright © 2012 Massachusetts Medical Society. All rights reserved.

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