ICD-11

A group of disorders involving apocrine glands and their associated follicular units. The most important of these is hidradenitis suppurativa.

Hidradenitis suppurativa is a chronic disease characterised by recurrent, painful, deep-seated, rounded nodules and abscesses due to follicular occlusion with secondary inflammation and destruction of the pilo-sebaceo-apocrine apparatus and extension to the adjacent subcutaneous tissue. Subsequent hypertrophic scarring and suppuration of apocrine gland-bearing skin (axillae, groins, peri-anal and perineal regions) are the main clinical features. Infection and hormonal influence are described but are not the primary pathogenetic factor: the exact aetiology remains unknown. Obesity is common and is associated with more severe disease. The main complications are fistulae, arthropathy, carcinoma and amyloidosis.

Hidradenitis suppurativa is a chronic disease characterized by recurrent, painful, deep-seated, rounded nodules and abscesses. Subsequent hypertrophic scarring and suppuration of apocrine gland bearing skin: axilla, groin, peri-anal and perineal regions are main features. The prevalence has been estimated at 1%, but is usually considered lower. Axillary, and inguinal involvement is more common in women; perineal form in men. Onset is usually after puberty, although it is most common during the third decade and may persist in old age. The disease tends to become chronic with subcutaneous extension leading to indurations, sinus, and fistula having a profound impact on the quality of life. The exact etiology remains unknown. The primary event is a follicular occlusion with secondary inflammation, infection and destruction of the pilo-sebaceo-apocrine apparatus and extension to the adjacent sub-cutaneous tissue. Although infection and hormonal influence are commonly observed, they are not the primary etiologic factor. Smoking may be a triggering factor. Obesity aggravates the discomfort. Differential diagnostic includes Crohn disease, nodular acne and furonculosis. The main complications are fistulae, arthropathy, carcinoma and amyloidosis. Treatment depends upon the stage of the disease. Early lesions are usually treated by medical therapy such as antibiotics for acute stage; long term antibiotics, systemic steroids, estrogens, anti-androgens, retinoïdes have been used as options for the chronic stage with limited success. Surgical treatment includes incision and drainage, followed by antibiotics for limited nodules, limited excisions. Total wide excision and healing with secondary intention or flaps and grafts is the only curative procedure in case of advanced disease.