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Hidradenitis suppurativa - Anogenital in
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Hidradenitis suppurativa - Anogenital in

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Contributors: Sarah N. Robinson MD, Susan Burgin MD, Lowell A. Goldsmith MD, MPH, Benjamin K. Fisher MD
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Synopsis

Hidradenitis suppurativa, or acne inversa, is a chronic destructive inflammatory disorder of the terminal follicular epithelium in apocrine gland-bearing regions. It is thought that follicular occlusion leads to trapping of follicular contents, rupture, and inflammation of the dermis, with bacterial superinfection in some cases. Hidradenitis suppurativa is more common in women and individuals of African descent.

The nodules of hidradenitis suppurativa are seen most commonly on the buttocks, breasts, and in the groin and axillae. Usually, the onset of the disease occurs soon after puberty, and patients typically report recurring "boils." Symptoms may include local pain and tenderness during a flare-up and arthralgias. Shaving, depilation, deodorants, and mechanical irritation can worsen this condition, but irritation of the skin is usually not a major factor.

Obesity and cigarette smoking are associated with hidradenitis suppurativa. Hidradenitis suppurativa and metabolic syndrome are strongly associated. Regional ileitis (Crohn disease) has a statistical association with hidradenitis suppurativa, while ulcerative colitis does not. A familial form of the disease has been supported by studies, including a molecular genetic study of 4 generations in a large Chinese family, through which a novel hidradenitis suppurativa locus on chromosome 1p21.1-1q25.3 was identified. Furthermore, many patients report a positive family history. Rare cases of hidradenitis suppurativa are associated with reticulate pigmented anomaly of the flexures (Dowling-Degos disease) and heterozygous mutations of PSENEN (gamma-secretase protein presenilin precursor). Hidradenitis suppurative-like lesions have been reported to very frequently occur during therapy with a gamma secretase inhibitor.

Hidradenitis suppurativa shares similar clinical features (severe inflammation, occlusion of the follicle, and scarring) with dissecting cellulitis of the scalp and acne conglobata. Collectively, these 3 conditions are referred to as the follicular occlusion triad, and more than 1 may occur in a given patient. Some consider the pilonidal sinus (pilonidal cyst) to be an additional member of this group.

With a prevalence of up to 1% in some population-based studies, hidradenitis suppurativa is a common disease.

Codes

ICD10CM:
L73.2 – Hidradenitis suppurativa

SNOMEDCT:
59393003 – Hidradenitis suppurativa

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Last Reviewed: 08/29/2016
Last Updated: 04/26/2018
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Hidradenitis suppurativa - Anogenital in
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Hidradenitis suppurativa : Axilla, Developed chronically lasting months to years, Inframammary fold of chest, Intertriginous, Pustule, Sinus, Inguinal region
Clinical image of Hidradenitis suppurativa
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