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Hailey-Hailey Disease (Familial Benign Pemphigus)

  • Carmen Maria Salavastru
  • George-Sorin TiplicaEmail author

Abstract

Hailey-Hailey disease is a rare autosomal dominant genetic disease due to mutations in the ATP2C1 gene. The defective gene induces the production of altered desmosomal components in the Golgi apparatus. The clinical diagnosis of Hailey-Hailey disease is based on the recurrent erythematous plaques with vesicles and fissures that occurred symmetrically in flexural areas. The lesions can become hypertrophic and malodorous. The histopathological elements are characteristic: acantholytic vesicles and a “dilapidated brick wall” structure of the epidermis. Hailey-Hailey disease has no specific treatment, and the patients have a poor quality of life. Local and systemic treatment includes antibiotics, antifungals and corticosteroids. Other treatments include: botulinum toxin injections, retinoids and laser CO2 ablation.

Keywords

Hailey-Hailey disease Blister ATP2C1 gene Acantholysis 

Further Reading

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Carmen Maria Salavastru
    • 1
  • George-Sorin Tiplica
    • 1
    Email author
  1. 1.2nd Clinic of DermatologyColentina Clinical Hospital – “Carol Davila” University of Medicine and PharmacyBucharestRomania

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