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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Hailey-Hailey disease


Other Names for this Disease
  • BCPM
  • Benign chronic pemphigus
  • Benign familial pemphigus
  • Familial benign pemphigus
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Overview



What is Hailey-Hailey disease?

How is Hailey-Hailey disease inherited?


What is Hailey-Hailey disease?

Hailey-Hailey disease is an inherited condition characterized by recurrent blisters seen primarily on the neck, groin, and axillae (armpit). Precipitating factors include heat, sweating, skin infection, and ultraviolet radiation. The disorder often becomes apparent after puberty, usually by the third or fourth decade, but symptoms can develop at any age.[1][2] Hailey-Hailey disease occurs due to a mutation in a specific gene (ATP2C1) that creates a protein that is essential for skin health.[1][3] There is no cure for this condition. Treatment is aimed at reducing symptoms and preventing flare-ups.[4] This condition is inherited in an autosomal dominant fashion.[3]
Last updated: 7/25/2011

How is Hailey-Hailey disease inherited?

Hailey-Hailey disease is inherited in an autosomal dominant fashion, which means that an affected person usually inherits the abnormal gene that causes the condition from one parent. A person that has an autosomal dominant condition has a 50% chance of passing it on to his or her children.[3]
Last updated: 7/25/2011

References
  1. Mauro T. Hailey-Hailey Disease. National Organization for Rare Disorders (NORD). 2009; http://rarediseases.org/rare-disease-information/rare-diseases/byID/1223/viewAbstract. Accessed 7/25/2011.
  2. Helm TN. Familial Benign Pemphigus (Hailey-Hailey Disease). eMedicine. 2010; http://emedicine.medscape.com/article/1063224-overview. Accessed 7/25/2011.
  3. Familial benign chronic pemphigus. Orphanet. 2006; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=2841. Accessed 7/25/2011.
  4. Lamb S. Hailey-Hailey disease. DermNet NZ. June 2011; http://www.dermnetnz.org/systemic/familial-pemphigus.html. Accessed 7/25/2011.