Hailey-Hailey disease pathology

Author: Dr. Abdurrahman Almurayshid, faculty member at Prince Sattam Bin Abdulaziz University, College of Medicine, Dermatology, Saudi Arabia. June 2015.

Hailey-Hailey disease pathology — codes and concepts

Hailey–Hailey disease, also called familial benign chronic pemphigus, is a blistering disease caused by mutations in the ATP2C1 gene, with autosomal-dominant inheritance. The ATP2C1 gene is vital for keratinocyte adhesion and differentiation. Usually the clinical presentation starts during the second or the third decade. The main affected areas are the skin folds, with moist fissured malodorous plaques and blisters. The course of disease is variable, and treatment is mainly symptomatic.

Histology of Hailey Hailey disease

In Hailey Hailey disease, acantholysis affects the whole epidermis, giving the classic description of the dilapidated brick wall (figures 1–4). The hair follicles are usually spared, typically, with acanthosis and dyskeratosis.

Hailey Hailey disease pathology

Special studies for Hailey Hailey disease

Direct immunofluorescence is applied to fresh tissue to exclude immunobullous disease, particularly pemphigus vulgaris.

Differential diagnosis of Hailey Hailey disease

Grover disease: usually more focal histologically.

Pemphigus vulgaris: positive immunofluorescence and more likely to involve hair follicles.


Related information



  • Dirk M. Elston, MD. Tammie Ferringer, MD. Dermatopathology Second Edition. Philadelphia. Elsevier; 2014
  • Jean L. Bolognia, Julie V. Schaffer, Karynne O. Duncan, and Christine J .Ko. Dermatology Essentials. Oxford. Elsevier; 2014.

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