Skin-nontumor / Clinical dermatology
Blistering disorders
Hailey-Hailey disease

Author: Mowafak Hamodat, M.D., MB.CH.B, MSc., FRCPC (see Authors page)

Revised: 14 June 2016, last major update July 2011

Copyright: (c) 2002-2016,, Inc.

PubMed Search: Hailey-Hailey disease

Cite this page: Hailey-Hailey disease. website. Accessed June 25th, 2017.
Definition / general
  • Autosomal dominant disorder of crusts, vesicles and erosions in intertriginous regions
  • Also called benign familial pemphigus
Clinical features
  • Lesions are frequently itchy and malodorous
  • Superinfection by Candida albicans, herpes virus and Staphylococcus aureus are frequent complications
  • Lesions occur secondary to mechanical trauma, stress and UV radiation
  • May be complicated by squamous cell carcinoma
  • Caused by mutations in ATP2C1 gene which encodes the protein hSPCA1; leads to acantholysis of skin (Nat Genet 2000;24:61)
Microscopic (histologic) description
  • Prominent suprabasal acantholysis, also acantholysis at all levels of epidermis, with upper keratinocytes held only loosely together
  • Acantholytic cells may retain some connections and resemble dilapidated bricks
  • Adnexal epithelium is usually spared
  • Occasionally dyskeratotic cells are seen resembling the corps ronds and grains of Darier’s disease
  • No / rare inflammation
Microscopic (histologic) images

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Suprabasal acantholysis