Dermatology - Hailey - Hailey Disease

Dermatology - Hailey - Hailey Disease 
Familial benign pemphigus, also known as Hailey-Hailey disease, is an uncommon genodermatosis with dominant inheritance.

The symptoms of Hailey-Hailey illness are erythematous, erosive, and oozing, with fissures and cracks that are restricted to the scrotum, axillae, nape of the neck, and submammary regions. The third and fourth decades are often when onset occurs. There are recurrent infections.

Lesions are characterized by tiny, microscopic flaccid vesicles on an erythematous background that eventually dissolve into degraded plaques with the distinctive, fissured appearance that has been described. There are hypertrophic vegetative lesions, crusting, and scaling.

Clinical presentation and family history are used to make the diagnosis, which is then verified by genetic testing. Intertrigo, candidiasis, and contact dermatitis are included in the differential.

The mainstay of treatment is antimicrobial therapy, which is used topically and systemically. Tetracyclines appear to function better than most other antibiotics systemically. One applies mupirocin topically.
Topical glucocorticoids hasten healing by suppressing the anti-inflammatory response. In extreme situations, carbon dioxide laser vaporization or dermabrasion result in scars that heal and are resistant to recurrence. As one ages, the condition becomes less problematic.