Hailey Hailey Disease (Familial Benign Pemphigus)

Hailey Hailey Disease (Familial Benign Pemphigus)

Infobox_Disease
Name = PAGENAME


Caption =
DiseasesDB = 29321
ICD10 = ICD10|Q|82|8|q|80
ICD9 = ICD9|757.39
ICDO =
OMIM = 169600
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
MeshID = D016506
Familial benign pemphigus originally was described by the Hailey brothers in 1939, [WhoNamedIt|synd|2081] [H. Hailey, H. Hailey. Familial benign chronic pemphigus. Report of 13 cases in four generations of a family and report of 9 additional cases in 4 generations of a family. Archives of Dermatology and Syphilology, Chicago, 1939, 39: 679-685.] and is commonly called Hailey Hailey disease.

Presentation

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 ATP2C1cite journal |author=Hu Z, Bonifas JM, Beech J, "et al" |title=Mutations in ATP2C1, encoding a calcium pump, cause Hailey-Hailey disease |journal=Nat. Genet. |volume=24 |issue=1 |pages=61–5 |year=2000 |month=January |pmid=10615129 |doi=10.1038/71701 |url=http://dx.doi.org/10.1038/71701] 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.

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.

References

External links

* [http://www.haileyhailey.com/ Hailey-Hailey site with links to Yahoo Support Group]


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