Hidradenitis suppurativa

Hidradenitis suppurativa or HS is a 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.

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. Flare-ups may be triggered by stress, hormonal changes (such as monthly cycles in women), humid heat, and clothing friction. 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 can be 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. It is possible that there is genetic predisposition to the disease. HS is not contagious.

Because HS is considered a rare disease, its incidence rate is not well known, but has been estimated as being between 1:24 (4.1%) and 1:600 (0.2%).^*

Other names for HS

Hidradenitis suppurativa has been referred to by multiple names in the literature, as well as in various cultures. Some of these are also used to describe different diseases, or specific instances of this disease. ^*

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:

a few minor sites with rare inflammation; may be mistaken for acne.
frequent inflammations restrict movement and require minor surgery.
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 controversial 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
sometimes linked with other auto-immune conditions
androgen dysfunction
genetic disorders that alter cell structure

Research currently implies that people with HS have an inappropriate immune response to the apocrine glands, possible triggered by a blocked gland, creating inflammation, pain, and a swollen lesion. HS is not caused by any bacterial infection -- any infection is secondary. Most cultures done on HS lesions come back negative for bacteria, so antibiotics should be used only when a bacterial infection has been confirmed by a physician.

Hidradenitis suppurativa is NOT contagious!

Severe Complications

Left undiscovered, undiagnosed, or untreated, the fistulas from severe stage-3 HS can lead to the development of squamous cell carcinoma in the anus or other affected areas.

Treatments

Treatments may vary depending upon presentation and severity of the disease. Due to the poorly-studied nature of this disease, the effectiveness of the drugs and therapies listed below is not yet clear, and patients should discuss all options with their doctor or dermatologist. A list of treatments that are effective for some patients is as follows.

changes in diet
warm compresses, baths (to induce drainage)
intralesional corticosteroid injections (to reduce inflammation)
incision and drainage or lancing
oral antibiotics (to treat inflammation and bacterial infection)
isotretinoin (Accutane®), a prescription-only oral acne treatment (benefits for HS are very controversial)
wide local excision (with or without skin grafting), or laser surgery
radiotherapy
anti-androgen therapy
sub-cutaneous injection or IV infusion of anti-inflammatory (anti-TNF-alpha) drugs such as infliximab (Remicade®)and etanercept (Enbrel®). This use of the drugs is not currently Food and Drug Administration (FDA) approved and is somewhat controversial, and therefore may not be covered by insurance.
Acitretin

External links

Dermatology | Inflammations

Hidradenitis suppurativa | Hidradenitis suppurativa | Hidradenitis suppurativa | Hidrosadenite

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