Hailey Hailey Disease (Familial Benign Pemphigus)
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Familial benign pemphigus originally was described by the Hailey brothers in 1939, as such is commonly called Hailey Hailey disease.
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 cycles of outbreaks.
The cause of the disease is a defect in the calcium pump ATP2C1 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.
Steroidal cremes have some effect during outbreaks, but do not stop them. Avoiding heat and friction of affected areas and keeping the area clean and dry work about as well.
Some have found relief in laser surgery that apparently burns off the top layer of the derma, allowing healthy non-affected skin to regrow in its place, but this method of treatment is new to the diease and lacks the tests of time.
Unfortunately, there is not much that really helps when the outbreaks come, except to suffer through them, "suffer" being the key word here, since the outbreaks are often extremely painful.
[edit] External Links
Hailey-Hailey site with links to Yahoo Support Group
Pemphigus is not Benign Familial Pemphigus, sometimes called Hailey-Hailey disease. Some medical books refer to Hailey-Hailey Disease as 'Benign Familial Pemphigus' but it is not true pemphigus. This is because Hailey-Hailey is not an autoimmune disease, it is genetic. There are some similarities with PV but Hailey-Hailey is not caused by an auto-antibody, as in PV. For more information see www.bad.org.uk/patients
