Hailey-Hailey disease

How is Hailey-Hailey disease diagnosed?

The diagnosis of Hailey-Hailey disease usually is made based on symptoms and family history. As it can be mistaken for other blistering skin conditions, a skin biopsy might be needed. Genetic testing is available to confirm the diagnosis, but is not required. ATP2C1 is the only gene known to be associated with Hailey-Hailey disease. Genetic testing for at-risk relatives and prenatal testing are also possible if the disease-causing mutation in the family is known.

Last updated on 05-01-20

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 features of the disease. Some people with Hailey-Hailey disease inherit the disease from a parent with the disease. Other cases are due to a new mutation in the gene and occur in people with no family history of the disease. 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.

Last updated on 05-01-20

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.

People with Hailey-Hailey disease 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. 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.

Topical medications (such as mild corticosteroid creams and topical antibiotics) may improve symptoms in milder forms. Cool compresses and dressings may also help. More severe cases may require systemic antibiotics (especially erythromycin and tetracycline) and/or stronger corticosteroid creams. Topical tacrolimus ointment can be used to control the disease, even without the use of topical corticosteroids.

When the disease does not improve with these treatments and it is widespread, additional treatment options may include:

Botulinum toxin, CO2 ablation laser, and dermabrasion are the most studied and have shown to be effective treatments. Some reports show that low-dose botulinum toxin type A injections should be given first in hard-to-treat cases because it is well-tolerated, effective, and has few or mild negative side effects.

Last updated on 05-01-20

Name: Foundation for Ichthyosis and Related Skin Types FIRST 2616 North Broad Street
Colmar, PA, 18915 , United States
Phone: +1-215-997-9400 Toll Free: 1-800-545-3286 Email: info@firstskinfoundation.org Url: http://www.firstskinfoundation.org/
Name: Hailey-Hailey Disease Society 515 Park St
Harrison, MI, 48625, United States
Email: http://haileyhailey.com/contact-us Url: http://haileyhailey.com
Prenatal tests March of Dimes. March 2015; Reference Link Familial benign pemphigus Genetic Testing Registry. Reference Link

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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