Hailey-Hailey disease, also known as familial benign chronic pemphigus, was first discovered by the Hailey brothers in 1939. Hailey-Hailey disease is a genetic disorder inherited among family members with a 50% chance of inheritance from an affected parent. It is most common in the late teenage years, twenties and thirties. The lesions are most commonly seen in the sides of the neck, armpit, and groin areas and under the breast. These patients may complain of a heavy odor and itching, which may be a social distress to patients’ lives.

The initial lesion may be a red, scaly area or a fluid filled blister which ruptures easily and becomes macerated or crusted. These lesions seem to come and go and leave no scars. The lesions may be localized to an area or may become widespread. Patients with Hailey-Hailey disease may also have nail manifestation called ‘longitudinal leukonychia’ which are longitudinal white bands on the fingernails.

Complications of Hailey-Hailey disease include risk of secondary bacterial, fungal, and viral infections, which may require antimicrobial agents. There is no cure for Hailey-Hailey disease since it is a genetic disorder. Treating patients for the symptoms does provide patients with relief and reduces the microbial infections.

The first part of treatment is to avoid friction and sweating by wearing lightweight clothes. Avoiding direct sun and using sunscreen may also be helpful. Applying soothing compresses followed by topical corticosteroids and topical antibiotics are often effective for localized lesions. Generalized lesions may require oral antibiotics in addition to the above treatments.

Resistant cases that fail topical treatment have responded well to the following; oral corticosteroids, dapsone, topical tacrolimus, and photodynamic therapy. Dermabrasion, laser treatment and surgical skin grafting can also be used in severe cases.

http://www.adv-derm.com