IPPF

Hailey-Hailey Disease (HHD) is not an autoimmune disease. It is genetic, and is seen intergenerationally, and often among siblings and extended family members. There is a 1:2 (50%) chance of inheriting the disorder from an affected parent.

There is a cell-cell detachment that resembles the process seen in true pemphigus, but there is no autoantibody causing the blisters. The detachment is found at the cell level and is exacerbated by friction and sweat. Loss of one functional copy of the gene coding for the protein SPCA1 (ATP2C1) causes Hailey-Hailey disease. This protein functions in skin cells to transport calcium and manganese ions. The gene has been mapped to chromosome 3, specifically 3q21-q24.

Because the disease is genetic, there is no cure. Treatment consists of oral antibiotics, anti-fungals and/or anti-virals to control secondary infection, astringents, cool compresses and dressings. Also, topical steroid creams applied have been known to help in most cases, although their long-term use is discouraged due to thinning of the already sensitive skin. Oral steroids and Dapsone are tried to help severe breakouts.

For further information concerning Hailey-Hailey, go to  www.haileyhailey.com .