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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
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is Hailey-Hailey disease?

How is Hailey-Hailey disease inherited?

How might Hailey-Hailey disease be treated?

What is Hailey-Hailey disease?

Hailey-Hailey disease is a hereditary blistering skin disease. Signs and symptoms include a painful rash and blistering in skin folds such as the armpits, groin, neck, under the breasts, and between the buttocks. Secondary bacterial infections are not uncommon. Symptoms are often worse in summer months due to heat, sweating and friction.[1] Hailey-Hailey disease is caused by mutations in the ATP2C1 gene and is inherited in an autosomal dominant manner.[2] Treatment focuses on reducing symptoms and preventing flares.[1]
Last updated: 5/14/2014

How is Hailey-Hailey disease inherited?

Hailey-Hailey disease is inherited in an autosomal dominant manner. This means that having only one mutated copy of the disease-causing gene in each cell is enough to cause signs or symptoms of the condition. Some people with Hailey-Hailey disease inherit the condition from an affected parent. Other cases are due to a new mutation in the gene and occur in people with no history of the condition in their family. When a person with an autosomal dominant condition has children, each child has a 50% (1 in 2) risk to inherit the mutated copy of the gene.[2]
Last updated: 5/14/2014

How might Hailey-Hailey disease be treated?

There is no specific treatment for Hailey-Hailey disease and management generally focuses on the specific symptoms and severity in each person. Affected people are encouraged to avoid "triggers" such as sunburn, sweating, and friction, and to keep the affected areas dry. Sunscreen, loose clothing, moisturizing creams, and avoiding excessive heat may help prevent outbreaks.[3] Trying to prevent bacterial, viral, and fungal infections in the affected areas is also important, and drugs used to treat or prevent these infections are commonly used.[3][4]

Topical medications (such as mild corticosteroid creams and topical antibiotics) may improve symptoms in milder forms.[3][4] Cool compresses and dressings may also help.[3] More severe cases may require systemic antibiotics and/or stronger corticosteroid creams.[3] Carbon dioxide laser treatment may be effective for severe forms. In very severe cases, surgery can be performed to remove the affected skin, but skin grafts are usually necessary to repair the wounds.[1]
Last updated: 5/14/2014

References
  1. Hailey-Hailey disease. DermNet NZ. December 29, 2013; http://www.dermnetnz.org/systemic/familial-pemphigus.html. Accessed 5/14/2014.
  2. Benign chronic pemphigus. Genetics Home Reference. May, 2013; http://ghr.nlm.nih.gov/condition/benign-chronic-pemphigus. Accessed 5/14/2014.
  3. Hailey-Hailey disease. NORD. August 2, 2012; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1223/viewAbstract. Accessed 5/14/2014.
  4. Familial benign chronic pemphigus. Orphanet. June, 2006; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=2841. Accessed 5/14/2014.


Other Names for this Disease
  • BCPM
  • Benign chronic pemphigus
  • Benign familial pemphigus
  • Familial benign pemphigus
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.