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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Look at other dictionaries:

  • Hailey-Hailey disease — (= familial chronic benign pemphigus) A blistering disease of the skin apparently due to a defect in epidermal cell junctions, even though apparently normal desmosomes and adherens junctions can be assembled. Transmitted as an autosomal dominant …   Dictionary of molecular biology

  • Hailey-Hailey disease — benign familial pemphigus …   Medical dictionary

  • Hailey-Hailey disease — Hai·ley Hai·ley disease (haґle haґle) [Hugh Edward Hailey, American dermatologist, 1909–1964; William Howard Hailey, American dermatologist, 1898–1967] benign familial pemphigus; see under pemphigus …   Medical dictionary

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  • pemphigus — pemphigoid /pem fi goyd /, adj. pemphigous, adj. /pem fi geuhs, pem fuy /, n. Pathol. any of several diseases, often fatal, characterized by blisters on the skin and mucous membranes. [1770 80; < NL < Gk pemphig (s. of pémphix) bubble + L us n.… …   Universalium

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  • benign familial pemphigus — a benign, autosomal dominant dermatitis due to mutation in the ATP2C1 gene (locus: 3q21 q24), which encodes a Ca2+ ATPase. It is characterized by recurrent bullae and vesicles, usually on the neck, axillae, groin, and other flexural and apposing… …   Medical dictionary

  • List of cutaneous conditions — This is an incomplete list, which may never be able to satisfy particular standards for completeness. You can help by expanding it with reliably sourced entries. See also: Cutaneous conditions, Category:Cutaneous conditions, and ICD 10… …   Wikipedia

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