National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Hidradenitis Suppurativa is not the name you expected.
Hidradenitis suppurativa (HS) is a chronic condition characterized by swollen, painful lesions, occurring in the armpit (axillae), groin, anal, and breast regions. This disease occurs due to obstruction of hair follicles and secondary infection and sometimes inflammation of certain sweat glands (apocrine glands). It is a progressive disease where single boil-like, pus-filled abscesses become hard lumps, then painful, deep-seated, often inflamed clusters of lesions with chronic seepage. Healing of affected areas is typically associated with progressive scarring (fibrosis). Formation of chronic epithelialized, sometimes interconnected, sinus tracts occur in severe disease. This often leads to the entrapment of perspiration and bacteria in the surrounding tissue, which causes the inflammation and infection. HS can be extremely painful and debilitating but is rarely life threatening; only occurring when the bacteria infection leads to an overwhelming systemic infection in an individual with a weakened immune system.
HS was once thought to be a rare disorder because only the most severe cases were reported. HS was first described in the medical literature by Velpeau in 1839 and was considered an extremely severe form of acne (acne inversa). It most often presents at puberty because changes in hair follicles triggered by the surge in sex hormones. The condition may also begin to occur in patients of any age after puberty, but rarely before. Cases vary in severity, but all require some treatment and management. For mild cases, home remedies may be all that is needed. For severe forms however, daily medication is often prescribed and radical surgery may eventually be recommended. Early diagnosis and treatment of HS is important because it can help manage symptoms and prevent new boils and lesions from forming.
Genetic and Rare Diseases (GARD) Information Center
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Hidradenitis Suppurativa Foundation, Inc
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NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases
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For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
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Last Updated: 3/15/2016
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