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Hailey-Hailey Disease

Last updated Sept. 18, 2018

Approved by: Krish Tangella MD, MBA, FCAP

Hailey-Hailey Disease is a rare genetic disease that causes painful blisters and rashes to form on the skin, often in the skin folds.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Familial Benign Chronic Pemphigus
  • Familial Benign Pemphigus
  • Gougerot-Hailey-Hailey's Benign Familial Pemphigus

What is Hailey-Hailey Disease? (Definition/Background Information)

  • Hailey-Hailey Disease is a rare genetic disease that causes painful blisters and rashes to form on the skin, often in the skin folds
  • The cause of Hailey-Hailey Disease is a genetic defect and the condition is inherited in an autosomal dominant manner
  • A treatment of the condition involves steroid therapy; the prognosis of Hailey-Hailey Disease is generally good, with proper treatment

Who gets Hailey-Hailey Disease? (Age and Sex Distribution)

  • Hailey-Hailey Disease skin lesions often begin during adolescence. The peak onset is seen between the period of 30-40 years
  • The disease is equally prevalent in both men and women

What are the Risk Factors for Hailey-Hailey Disease? (Predisposing Factors)

Hailey-Hailey Disease is a genetic disorder, and thus a family history of this disease is a risk factor.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Hailey-Hailey Disease? (Etiology)

  • Hailey-Hailey Disease is an autosomal dominant genetic condition
  • It is caused by missing one of the two copies of the ATP2C1 gene on chromosome 3, which encodes the protein hSPCA1
  • Approximately 30% of the cases, are caused by random mutations during fetal development

Autosomal dominant: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly function gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children, who do not inherit the abnormal gene, will not develop the condition or pass it on to their offspring.

What are the Signs and Symptoms of Hailey-Hailey Disease?

Hailey-Hailey Disease signs and symptoms include:

  • The individuals develop painful blisters and rashes in the skin folds, which often recur
  • The body regions where these appear, include the underarm or groin area

How is Hailey-Hailey Disease Diagnosed?

A diagnosis of Hailey-Hailey Disease may involve:

  • The physician undertakes a physical examination and study of family history of the individuals with the condition
  • The Hailey-Hailey Disease is most often diagnosed, by studying the clinical features of the disease
  • Skin biopsy, which is examined by a pathologist under a microscope

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Hailey-Hailey Disease?

The possible complications from Hailey-Hailey Disease include:

  • The skin lesions may become infected, or problematic by heat and rubbing (friction)
  • In very rare instances, the skin lesions may develop into squamous cell carcinomas

How is Hailey-Hailey Disease Treated?

  • There is no cure for Hailey-Hailey Disease, but agents, such as intravenous or topical steroids, are most often used to reduce inflammation and provide symptomatic relief
  • Other drugs that have been shown to treat the symptoms of the disease include alefacept, dapsone, methotrexate, thalidomide, etretinate, and cyclosporine
  • Antibiotics and antifungals may be used, if there is a risk or an indication of infection

How can Hailey-Hailey Disease be Prevented?

  • Hailey-Hailey Disease is a complex disease believed to involve both, one's genetic predisposition and certain environmental factors
  • Currently, there are no definitive methods to prevent Hailey-Hailey Disease

What is the Prognosis of Hailey-Hailey Disease? (Outcomes/Resolutions)

  • There is no cure for Hailey-Hailey Disease genetic disorder
  • Treatment with the medications (discussed earlier) may often lead to a resolution of the symptoms; but, recurrent episodes are generally observed
  • Individuals with Hailey-Hailey Disease are also often affected by other contact allergies
  • Overall, with treatment, the prognosis for Hailey-Hailey Disease is good

Additional and Relevant Useful Information for Hailey-Hailey Disease:

  • Hailey-Hailey Disease was originally described by the brothers, Hugh Edward Hailey and William Howard Hailey, in 1939
  • The disease is sometimes referred to as Gougerot-Hailey-Hailey Syndrome, in order to acknowledge the contributions of Henri Gougerot, in 1933

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: May 20, 2014
Last updated: Sept. 18, 2018