Elsevier

Dermatologic Clinics

Volume 28, Issue 4, October 2010, Pages 779-793
Dermatologic Clinics

Hidradenitis Suppurativa

https://doi.org/10.1016/j.det.2010.07.003Get rights and content

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Definition

HS is defined clinically by its various features and by its chronicity. It is a recurrent disease, classically, but not exclusively, in the apocrine gland–bearing areas of the skin, manifesting as painful, recurrent, deep-seated, inflamed nodules that can result in abscesses, sinuses, and varying degrees of chronic draining sinus tracts with scarring, disfigurement, and disability.

The Second International HS Research Symposium (San Francisco, March 2009) adopted the following consensus

Diagnostic criteria

The Second International HS Research Symposium also adopted the following diagnostic criteria:

  • 1.

    Typical lesions: either deep-seated painful nodules (blind boils) in early primary lesions or abscesses, draining sinuses, bridged scars, and tombstone open comedones in secondary lesions

  • 2.

    Typical topography: axillae, groin, genitals, perineal, and perianal region, buttocks, infra- and intermammary folds

  • 3.

    Chronicity and recurrences.

These 3 criteria must be met to establish the diagnosis.6

HS is recognized

Differential diagnosis

HS has an extensive differential diagnosis (Box 2).8 The appearance, age of onset, typical locations, poor response to antibiotics, and lack of signs of systemic sepsis can all help distinguish this condition, so the diagnosis should be obvious. There are few conditions that cause recurrent abscesses and sinus tract formation in the intertriginous skin areas, such as Crohn disease, ulcerative colitis, and granuloma inguinale. The most common differential diagnoses are the follicular pyodermas:

Prevalence and epidemiology

HS is a common, forgotten, and orphaned disease. It is often not recognized by physicians, even dermatologists, and the results are devastating for patients. HS is mistakenly referred to as a rare disease. The global prevalence has been reported as between 1% and 4%, depending on the definitions used.9 Jemec10 reported a prevalence of 4% in a series of self-reported cases, with a high prevalence in young adults that was confirmed in another study.11 Revuz reported the prevalence in persons 55

Etiology

Several factors are related to the development of HS.

Clinical Description

HS classically occurs in the early 20s. The onset can be insidious, with apparently random small lesions that develop as a red, indurated papule, pustule, or nodule that may resolve without leaving any mark (Box 3). The lesions may develop in only 1 area, or several areas over weeks or months. The discomfort varies from vague itching to mild to moderate pain. In some cases, the onset can be severe, even frightening, with large, deep, painful lesions that result in restriction of activities. Too

Clinical course

The mean age of onset is 22.1 years and HS lasts about 19 years.7 It can remit or partially remit with pregnancy and breastfeeding. The course for patients can be variable. It may be intermittent and benign, with mild but chronic painful disease, acute exacerbations, premenstrual flares, and usually resolution after menopause. It may remit for weeks to months, lesions may flare continuously or only intermittently, and marked involvement may occur in only 1 area with a long deep-draining sinus

Hurley stages

In the recent report by Canoui-Poitrine and colleagues,16 68.2% of patients are Hurley stage I, 27.6% Hurley stage II, and 3.9% Hurley stage III (Box 4). To more accurately assess outcome variables related to HS treatment, the Sartorius score has been developed to monitor patients. It is to act as a complimentary system to the Hurley classification, and is presently undergoing evaluation as a research tool.33

Morbidity/quality of life

It is usual for patients with severe disease to be unemployable and socially isolated because of the painful, draining, and malodorous lesions.34 HS has a profoundly negative effect on patients, not only at a physical level but socially and economically. von der Werth and Jemec35 studied 144 patients with HS with a dermatology life quality index and found a higher morbidity index than in mild to moderate psoriasis or alopecia. The high score was related to pain, malodorous discharge, intimate

Associated diseases

HS has been associated with severe acne (acne conglobata), dissecting cellulitis of the scalp, and pilonidal cysts (Box 5).40, 41 Pilonidal cysts have been associated with HS in up to 30% of cases. Acne vulgaris is only found with HS in about 10% of women and 30% of men but a history of long-lasting, scarring acne was found in 23% of women and 44% of men. Dissecting cellulitis of the scalp is rare (1%); Revuz5 has never seen the follicular triad in 500 patients he has followed.

Keratitis-ichthyosis-deafness Syndrome

Two rare pigmentary disorders have been associated with HS. Dowling-Degos disease is a genetic condition with flexural pigmented macules in the axilla, neck, and groin. Kitamura disease has reticulate acropigmentation on hands and feet. The association of these conditions with HS is soft.9 There are many reports of rheumatologic associations or complications of HS, especially in African American men. This seems to be related to chronic inflammation.42, 43, 44, 45 SAPHO syndrome has been

Complications

HS is associated with several complications, especially in long-term untreated disease (Box 6). Fistulae are rare and associated with long-standing disease.8 The most common complications are anal and perianal fistulae, and it is speculated that these occur because of repeated or persistent abscess formation in the anal glands between the internal and external sphincter.54 Arthropathy can be associated with or can complicate HS (see earlier discussion). In late disease in the genital and

Treatment

There is no single effective treatment or cure for HS. The only permanent cure has been reported for very severe HS (Hurley III) with wide surgery. Most patients with HS require a combination of medical and surgical strategies, beginning with gentle atraumatic care.

General care for HS is based on clinical experience:

  • Gentle local hygiene. Washing with a mild nonsoap cleansing bar is all that is necessary. If there is odor then an antiseptic cleanser with triclosan could be used. Washing should

The future

Extensive information is available from the Hidradenitis Suppurativa Foundation at www.HS-foundation.org and the International Society for the Study of Vulvovaginal Disease at its educational site www.issvd.org, and the subject is regularly updated at www.UpToDate.com.

Patients with HS (and their physicians, surgeons, and dieticians) need to understand this disease to develop realistic expectations and to work with each other for the best outcome. They need to know that HS is not contagious and

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