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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


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

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.
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1 question(s) from the public on Hailey-Hailey disease have been answered. See questions and answers. You can also submit a new question.

Basic Information

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • Genetics Home Reference contains information on Hailey-Hailey disease. This website is maintained by the National Library of Medicine.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Hailey-Hailey disease. Click on the link to view a sample search on this topic.