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Hidradenitis suppurativa

Hidradenitis suppurativa or HS is a poorly studied skin disease that affects areas bearing apocrine sweat glands and hair follicles; such as the underarms, groin and buttocks, and under the breasts in women. more...

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Overview

The disease manifests as clusters of chronic abscesses or boils, sometimes as large as baseballs, that are extremely painful to the touch and may persist for years with occasional to frequent periods of inflammation, culminating in drainage, often leaving open wounds that will not heal. Drainage provides some relief from severe, often debilitating, pressure pain. These flare-ups are often triggered by stress, hormonal changes, or humid heat. Persistent lesions may lead to the formation of sinus tracts, or tunnels connecting the abscesses under the skin. At this stage, complete healing is usually not possible, and progression of the disease in the area is inevitable. Occurrences of bacterial infections and cellulitis (deep tissue inflammation) are likely at these sites. HS pain is difficult to manage.

HS often goes undiagnosed for years because patients are too ashamed to speak with anyone. When they do see a doctor, the disease is frequently misdiagnosed or prescribed treatments are ineffective, temporary and sometimes even harmful. There is no known cure nor any consistently effective treatment.

Although HS is considered a rare disease, its incidence rate is estimated as high as 1 in 300 people.

Other names for HS

  • Acne conglobata
  • Acne Inversa (AI)
  • Apocrine Acne
  • Apocrinitis
  • Fox-den disease
  • Hidradenitis Supportiva
  • Pyodermia sinifica fistulans
  • Velpeau's disease
  • Verneuil's disease

Stages

HS presents itself in three stages:

  1. a few minor sites with rare inflammation; may be mistaken for acne.
  2. frequent inflammations restrict movement and require minor surgery.
  3. inflammation of sites to the size of golf balls, or sometimes baseballs; scarring develops, including subcutaneous tracts of infection (see fistula). Obviously, patients at this stage may be unable to function.

Causes

As this disease is poorly studied, the causes are controvertial and experts disagree. However, potential indicators include:

  • post-pubescent
  • females are more likely than males
  • genetic predisposition
  • plugged apocrine (sweat) gland or hair follicle
  • excessive sweating
  • bacterial infection
  • linked to some immunodeficiency conditions
  • androgen dysfunction
  • genetic disorders that alter cell structure

Research currently implies that people with HS have a tendency towards clogged apocrine glands, which may then become infected with bacteria commonly present on the skin, and the immune system overreacts with excessive inflammation. Attempted treatments can target any of these three aspects of HS.

Read more at Wikipedia.org


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Surgical excision best tx for hidradenitis suppurativa, expert says - Anogenital Disease
From OB/GYN News, 4/1/03

HOUSTON -- Surgery remains the treatment of choice for patients plagued by anogenital hidradenitis suppurativa, Dr. Peter J. Lynch said at a conference on vulvovaginal diseases sponsored by Baylor College of Medicine.

Surgical excision is the only approach that truly eliminates the disorder's painful, inflamed, disfiguring nodules characteristic of this disorder, he said.

Antibiotics are of limited efficacy. Corticosteroids administered systemically for 5-7 days relieve inflammation only temporarily Dermatologists rarely perform this type of surgery, so often the task falls to the gynecologist or gynecologic surgeon.

"It's better to do a narrow excision and leave active disease on the margins than a more extensive procedure because the disease will recur at the surgical site anyway," Dr. Lynch said.

It's important to warn patients that the condition usually recurs, so they'll probably need surgery more than once. The nodules have a significant impact on quality of life because they hurt, rupture, leave scars, rub against clothing, and interfere with sexual intercourse. Most patients are "deliriously happy" to have them removed with surgery and generally are willing to undergo surgery again later if needed, said Dr. Lynch, professor emeritus of dermatology at the University of California, Davis.

Hidradenitis suppurativa occurs in hair follicles above apocrine ducts in the "milk line"--the axillas, breasts, central abdomen, and anogenital area--that become blocked and cannot normally discharge accumulated keratinous and bacterial debris, which builds up until the duct and the follicle rupture and spew it into the surrounding tissue, setting up an inflammatory foreign-body reaction.

The lesions, which are usually 14 cm in diameter, may also appear on the upper inner thighs and over the buttocks. Drainage of purulent material may continue for days or even weeks, and some patients develop regional lymphadenopathy, marked by mild fever, arthralgia, and malaise.

Furunculosis is the differential diagnosis, but in that condition the lesions are isolated and scattered and don't occur in the milk line. When in doubt, look for the diagnostic hallmark of hidradenitis suppurativa: twin comedones in the affected area, the result of an abnormal Y-shaped bifurcation of the follicular outlet.

That bifurcation is one of the factors that makes it difficult for the follicle to dispel its accumulated debris. Heat and sweat retention and friction caused by rubbing of clothing or skin also contribute to the occlusion. Obese women and African Americans seem to be at especially high risk.

Untreated, the lesions may heal but new ones develop, and the condition remains active until scarring eliminates all the involved follicles. In severe cases, frank genital mutilation may occur, he said.

Patients with the disease also frequently have cystic acne, which occurs through an analogous process. Rare cases of squamous cell carcinoma have been associated with long-standing disease.

COPYRIGHT 2003 International Medical News Group
COPYRIGHT 2003 Gale Group

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