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Hidradenitis Suppurativa
Updated: Mar 4, 2009
Introduction
Background
Hidradenitis suppurativa is an annoying chronic condition characterized by swollen, painful, inflamed lesions in the axillae, groin, and other parts of the body that contain apocrine glands. The disease is a chronic acneiform infection of the cutaneous apocrine glands that also can involve adjacent subcutaneous tissue and fascia. The hallmark of the disease is sinus tracts (which can become draining fistulas) in the apocrine gland body areas. Velpeau first described the condition in 1839.
Pathophysiology
The condition has classically been thought to occur when apocrine gland outlets become blocked by perspiration or are unable to drain normally because of incomplete gland development. Secretions trapped in the glands force perspiration and bacteria into surrounding tissue, causing subcutaneous induration, inflammation, and infection. However, more recent studies have indicated that hidradenitis suppurativa is caused by follicular occlusion first, which, in turn, occludes the apocrine glands and causes perifolliculitis. Therefore, it is actually a disorder of the terminal follicular epithelium located in the apocrine gland-bearing skin areas, which may better be termed as acne inversa.1
Hidradenitis suppurativa is confined to areas of the body that contain apocrine glands. These areas are the axillae, areola of the nipple, groin, perineum, circumanal, and periumbilical regions.
Often, patients with hidradenitis suppurativa also are afflicted with acne, pilonidal cysts, and chronic scalp folliculitis; thus, giving rise to the term follicular occlusion tetrad.
For further information, see Hidradenitis Suppurativa.
Frequency
United States
The problem is somewhat common, thought to occur in 1-2% of the population, but the precise incidence and prevalence are unknown.
International
A Danish study noted the prevalence of hidradenitis suppurativa to be in 4% of women.2
Mortality/Morbidity
Hidradenitis suppurativa is painful and can be disabling but is rarely fatal, except when it progresses to overwhelming systemic infection in an immunocompromised patient. Extensive disease can prevent patients from performing normal work functions and from engaging in normal social activities. In some patients, especially those with severe disease, the condition creates significant psychological problems, particularly regarding sexual relationships.
Race
Ingrown hairs are a predisposing factor, thus an increased incidence of the disease occurs in patients with tightly curled hair.
Sex
The incidence of hidradenitis suppurativa is greater in females than in males, thought to be in the range of 4:1 or 5:1. Flare-ups have been associated with menses, with a higher incidence in females with shorter cycles and more days of bleeding during the period.3
Age
Hidradenitis suppurativa does not present prior to puberty because the apocrine glands are inactive until triggered by a surge in sex hormones. The condition may be observed in patients of any age after puberty.
Clinical
History
The most common presentation is that of painful, tender, firm, nodular lesions under the arms.
- The nodules may open and drain pus spontaneously. Nodules will heal slowly, with or without drainage, over 10-30 days.
- In typical cases, nodules recur at least several times yearly.
- In severe cases, the patient may suffer a constant succession of new lesions forming as soon as old lesions heal.
- Excessive heat, perspiration, tight clothing,4 and obesity4 seem to aggravate the condition. Studies also show that cigarette smoking is a precipitator of the condition.
- Remissions may last months or years.
Physical
- The patient may present in considerable pain, with multiple red, hard, raised nodules in areas where apocrine glands are concentrated.
- Affected areas may include the axillae, periareolar, intermammary zones, pubic area, infraumbilical midline, gluteal folds, genitofemoral areas (top of the thighs in genital area), and the perianal region.
- As suppuration progresses, surrounding cellulitis may be present. Chronic recurrences result in palpable thick sinus tracts under the skin, which may turn into draining fistulas.
- The patient may present with a chronic condition in which the multiple nodules have coalesced and are surrounded by a fibrous reaction. This results in scarred and unsightly appearance of the area.
- Hidradenitis suppurativa may resemble recurrent bacterial folliculitis and furunculosis.
Causes
- A genetic predisposition to hidradenitis suppurativa likely exists, with one study noting that 38% of patients had a relative with hidradenitis.5
- Excessive perspiration, often observed in athletes and the obese, may contribute to clogging of the apocrine glands.
- Disease activity may be related to stress and to cigarette smoking.
- Hidradenitis may be observed as a primary condition without any obvious cause, but it may be observed in association with the following conditions:
- Crohn disease
- Irritable bowel syndrome
- Down syndrome
- Certain forms of arthritis
- Graves disease or Hashimoto thyroiditis
- Sjögren syndrome
- Herpes simplex
More on Hidradenitis Suppurativa |
Overview: Hidradenitis Suppurativa |
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| References |
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References
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Williams ST, Busby RC, DeMuth RJ. Perineal hidradenitis suppurativa: presentation of two unusual complications and a review. Ann Plast Surg. May 1991;26(5):456-62. [Medline].
Further Reading
Keywords
hidradenitis suppurativa, acne inversa, follicular occlusion, acne, pilonidal cysts, chronic scalp folliculitis, spiradenitis, apocrine glands, sweat glands, chronic acneiform infection, nodular lesions, cellulitis, excessive perspiration, Crohn disease, irritablebowel syndrome, Down syndrome, arthritis, Graves disease, Hashimoto thyroiditis, Sjögren syndrome, herpes simplex, Crohn's disease, Sjögren's syndrome
Overview: Hidradenitis Suppurativa