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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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Marjolin's ulcer in chronic hidradenitis suppurativa
From American Family Physician, 5/1/05 by Victoria A. Crain

TO THE EDITOR: Hidradenitis suppurativa is a chronic inflammatory disease of the apocrine glands that most commonly involves the perianal, perineal, axillary, and inframammary regions. (1) The etiology is poorly understood, with a genetic component, hormonal influence, and obesity being linked to the expression. (2)

Characteristic findings include dilated keratin-filled pores, scarring and sinus tract formation, malodorous discharge, and inflammation with secondary infection. (3) Medical management consists of cleaning the areas, application of mild topical steroids with the concurrent use of systemic antibiotics, (4) topical antibiotics, (5) and intralesional steroids. Rarely, with chronic hidradenitis suppurativa, Marjolin's ulcer (a squamous cell carcinoma) develops. (6) We report two cases of hidradenitis suppurativa in which squamous cell carcinoma developed after a latent period of 20 years and 35 years, respectively, despite repeated local excisions.

Case 1: A 51-year-old white man with a 35-year history of hidradenitis suppurativa presented with an ulcerated mass over the left scapular region (see accompanying figure). He previously had multiple local excisions. The entire mass was excised. After one month, the patient developed an abscess adjacent to the incision site, which proved to be squamous cell carcinoma on biopsy. Chemotherapy and radiotherapy were initiated. However, the tumor rapidly spread locally and a wider excision with latissimus dorsi flap reconstruction was performed. Unfortunately, the carcinoma spread rapidly to the axilla and neck, and the patient soon developed metastases in the lungs and spinal cord. He refused spinal decompression and died two weeks later.

Case 2: A 44-year-old black woman presented with a 20-year history of hidradenitis suppurativa involving vulvar, perianal, perineal, and inframammary regions. She had undergone more than 20 surgical procedures including skin grafts to ablate the disease. Examination revealed a large abscess at the level of the vaginal introitus and an extensive ulcerated fungating lesion involving the right labial crural fold. Her entire perianal region was ulcerated and scarred, and vaginal examination was not possible. Multiple biopsies were performed revealing infiltrating, moderately differentiated squamous cell carcinoma of the right labia, distal vagina, and urethra, with satellite lesions on the mons and right medial thigh.

Magnetic resonance imaging and computed tomography (CT) of the abdomen and pelvis revealed extensive bilateral inguinal, pelvic, and periaortic lymphadenopathy extending to a level inferior to the renal arteries. CT also revealed metastases of the squamous cell carcinoma to the right pubic bone. The patient continued to refuse operative management, instead opting for palliative high-dose radiation to control bleeding. She died from the carcinoma in six months.

Both of these patients presented with widespread metastases and a resultant terminal outcome. It would appear that a clear association between chronic hidradenitis suppurativa and squamous cell carcinoma exists. Because of the poor prognosis associated with squamous cell carcinoma, we advocate wide excision of hidradenitis suppurativa lesions when other treatments have failed and recommend biopsy of all suspicious hidradenitis suppurativa lesions.

VICTORIA A. CRAIN, M.D.

SALIL GULATI, M.D.

SATYANARAYANAN BHAT, PH.D.

STEPHEN M. MILNER, M.D.

The Plastic Surgery Institute

Southern Illinois University School of Medicine

747 N. Rutledge St.

Springfield, IL 62794

REFERENCES

(1.) Slade DE, Powell BW, Mortimer PS. Hidradenitis suppurativa: pathogenesis and management. Br J Plast Surg 2003;56:451-61.

(2.) Yu CC, Cook MG. Hidradenitis suppurativa: a disease of follicular epithelium, rather than apocrine glands. Br J Dermatol 1990;122:763-9.

(3.) Dictionnaire de medicine, un Repertoire general des sciences medicales considerees sous le rapport theorique [in French]. 2d ed. Paris: Bechet Jne., 1839:91.

(4.) Brenner DE, Lookingbill DP. Anaerobic microorganisms in chronic suppurative hidradenitis. Lancet 1980;2:921-2.

(5.) Clemmensen OJ. Topical treatment of hidradenitis suppurativa with clindamycin. Int J Dermatol 1983;22:325-8.

(6.) Lin MT, Breiner M, Fredricks S. Marjolin's ulcer occur-ring in hidradenitis suppurativa. Plast Reconstr Surg 1999;103:1541-3.

COPYRIGHT 2005 American Academy of Family Physicians
COPYRIGHT 2005 Gale Group

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