Hailey-Hailey disease begins as tiny, flaccid vesicles that may coalesce into bullae on normal or erythematous skin. The fragile bullae tend to rupture, leaving moist erosions and crusts. Vegetative, eroded plaques may form in established lesions. The eroded plaques tend to spread peripherally with central clearing, resulting in a circinate and/or serpiginous morphology. Microbial colonization and infection are common and may render malodorous lesions. Segmental involvement along the lines of Blaschko due to mosaicism has been reported. Pruritus and pain may be associated with the lesions, especially in high-friction areas.
Hailey-Hailey disease is caused by mutations in the ATP2C1 gene on chromosome 3. This gene encodes a Ca2+/Mn2+ ATPase protein. The abnormal protein alters calcium signaling and thereby leads to acantholysis.
The course is chronic, but severity tends to wax and wane. Heat, humidity, friction, and ultraviolet (UV) exposure are known to exacerbate this disease.
Q82.8 – Other specified congenital malformations of skin
79468000 – Familial benign pemphigus
Differential Diagnosis & Pitfalls
- Candidiasis – beefy red; look for satellite pustules
- Inverse psoriasis – look for associated nail changes such as pitting
- Dermatophyte infection – rarely bullous (see tinea cruris and tinea corporis)
- Pemphigus vegetans
- Pemphigus foliaceus
- Linear IgA bullous dermatosis
- Mucous membrane pemphigoid
- Granular parakeratosis – mostly seen in female axillae; brownish red papules coalesce to plaques
- Extramammary Paget disease
- Amicrobial pustulosis of the folds
- Papular acantholytic dyskeratosis (see Darier disease and transient acantholytic dermatosis)