Hailey-Hailey Disease Symptoms

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    Early Symptoms

    • The earliest signs of Hailey-Hailey disease, according to the AOCD, are likely to be red, scaly areas or fluid-filled blisters. Blisters may rupture easily and develop a crusty covering. The AOCD reports that Hailey-Hailey lesions "seem to come and go and leave no scars." The areas of initial irritation tend to heal first near the center but continue to spread out on the periphery.

    Where to Look for Symptoms

    • Hailey-Hailey lesions are seen most often in body folds, such as the groin, armpits, neck and beneath the breasts. The disorder occasionally involves the fingernails, showing up as longitudinal white bands, a symptom that is known as "longitudinal leukonychia." Hailey-Hailey lesions are very rare on mucosal tissue.

    Lesion-Related Symptoms

    • Lesions may cause intense itching and/or pain and produce a foul odor. Crusted lesions in moist areas of the body, most notably the groin, sometimes develop fissures, which can be extremely painful and open the patient to the possibility of secondary infection. The symptoms of Hailey-Hailey are often misdiagnosed as impetigo, oropharyngeal candidiasis (thrush), tinea (jock itch) and other skin conditions that cause blistering.

    Treatment and Complications

    • There is no cure for Hailey-Hailey disease. Treatment is limited to medications that relieve symptoms and the avoidance of conditions that may trigger outbreaks. Relief is often obtained through the use of topical corticosteroid and/or antibiotic creams and ointments, oral antibiotics and laser or phototherapy. Triggering conditions include heat, sweating, sunburn and friction. Possible complications, according to the AOCD, include secondary bacterial, fungal or viral infections.

    Genetic Nature of the Disorder

    • The website of the Hailey-Hailey Disease Society explains that the defective gene responsible for the skin disorder is carried on an autosomal chromosome (not one of the X or Y sex-determining chromosomes) and is dominant. This defect interferes with "keratinocyte adhesion," which is the normal tendency of the skin cells to adhere to one another. The result is weaker skin.

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