Hailey–Hailey disease
Hailey–Hailey disease (pronounced: HAY-lee HAY-lee disease), also known as familial benign chronic pemphigus, is a rare, chronic skin condition. It is characterized by recurrent blisters and erosions, primarily in areas of the skin that rub together.
Etymology
The disease is named after the American dermatologists, the Hailey brothers, who first described the condition in 1939. The term "pemphigus" comes from the Greek word "pemphix", meaning "blister" or "pustule".
Symptoms
The primary symptom of Hailey-Hailey disease is the development of blisters and erosions on the skin, particularly in areas where the skin rubs together, such as the armpits, groin, neck, and under the breasts. These lesions can be painful and may become infected. Other symptoms can include itching, crusting, and a foul odor.
Causes
Hailey-Hailey disease is caused by a mutation in the ATP2C1 gene. This gene provides instructions for making a protein that helps regulate the levels of calcium within cells. The mutation disrupts this regulation, leading to the formation of blisters and erosions on the skin.
Diagnosis
Diagnosis of Hailey-Hailey disease is typically based on the characteristic symptoms and confirmed by a skin biopsy. Genetic testing can also be used to identify the ATP2C1 gene mutation.
Treatment
There is currently no cure for Hailey-Hailey disease, but treatments can help manage the symptoms. These may include topical corticosteroids, antibiotics to treat infections, and laser therapy to reduce the formation of new blisters.
See also
External links
- Medical encyclopedia article on Hailey–Hailey disease
- Wikipedia's article - Hailey–Hailey disease
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