Author: Dr Stanley Leong, Dermatology and Paediatric Registrar (2023)
Reviewing dermatologist: Dr Ian Coulson (2023)
Edited by the DermNet content department
A 30-year-old woman presented with a four-year history of moist, malodorous plaques, with erosions and fissures in her left armpit and right breast fold.
Her mother has a similar skin condition.
On examination, she had an erythematous, eroded plaque in her left axilla, and a chronic submammary lesion with erosions under her right breast.
This is Hailey-Hailey disease. It is also called benign familial pemphigus. It is an autosomal dominant genodermatosis.
It is due to a mutation in the ATP2C1 gene, causing dysfunction of a Golgi-associated protein and interfering with cellular calcium signalling.
The onset of disease is usually during the second or third decade, but sometimes delayed into the fourth or fifth decade.
It mainly affects the major body folds such as the axillary, inframammary, and groin folds, and sometimes the trunk.
Widespread separation of the epidermal keratinocytes (acantholysis) resulting in a “dilapidated brick wall” appearance. There may be mild dyskeratosis.
Direct immunofluorescence is negative in Hailey-Hailey disease.
There is currently no cure for Hailey-Hailey disease. Avoidance of triggers and symptom management are the mainstay of management.
General measures include wearing lightweight clothing, antiseptic washes or bleach baths, and humectants.
Symptom management options may include intermittent topical or intralesional steroids, topical calcineurin inhibitors, botulinum toxin injections, and low dose naltrexone.
