Hailey Hailey disease
Overview
Hailey-Hailey disease, or familial benign pemphigus, was originally described by the Hailey brothers in 1939.[1][2] It is a genetic disorder that causes blisters to form on the skin.
Symptoms
It is characterized by outbreaks of rashes and blisters in the skin, usually in the folds of the skins, but also often over large areas of the body. The painful blisters break and sometimes become infected and raw, with new blisters forming over raw skin in a sometimes seemingly unending cycle of outbreaks.
Causes
The cause of the disease is a defect in the calcium pump ATP2C1[3] gene which is located on chromosome 3. This causes the cells of the skin to not adhere together properly, causing the blisters and rashes. There is no cure.
Diagnosis
Usually Hailey-Hailey disease is diagnosed by its appearance and the family history, but it is often is mistaken for other skin problems. Impetigo, thrush, tinea (jock itch) and other blistering conditions look similar. Diagnosis may require a skin biopsy. The histology is characteristic, with layers of detached skin cells (‘acantholysis’). Unlike pemphigus vulgaris, the immunofluorescence test for antibodies is negative.
Treatment
Topical steroid preparations have some effect during outbreaks, but do not stop them. Secondary bacterial, fungal and/or viral infections are common and may exacerbate an outbreak. Avoiding heat and friction of affected areas and keeping the area clean and dry work about as well. Some have found relief in laser resurfacing that burns off the top layer of the epidermis, allowing healthy non-affected skin to regrow in its place. Reducing sweat production is helpful in many cases. Some patients have found that outbreaks are triggered by certain foods, hormone cycles and stress.