Causes

Hailey-Hailey disease, or familial benign pemphigus is an autosomal dominant disorder, is hypothesized to result from a genetic defect in a calcium pump protein - ATP2C1.

This causes the cells of the skin to not adhere together properly, causing the blisters and rashes.

Signs and symptoms

• Vesicles and erythematous plaques with overlying crusts typically occur in the genital area, as well as the chest, neck, and axillary areas.
• Common sites include the armpits, groins, and neck, under the breasts and between the buttocks.
• Burning and itching is associated the eruption, and a malodorous discharge occurs in some cases as a result of secondary infection.
• The lesions tend to come and go and leave no scars. As the lesions get bigger the centre clears leaving a typical ring shape.
• Multiple asymptomatic longitudinal white bands or ridges on the fingernails
• Heat, sweating and friction often exacerbates the disease, and most patients have worsening of symptoms during the summer months

Tests and diagnosis

Diagnosis by skin biopsy

Treatment

• Avoiding triggering factors such as sunburn, sweating and friction helps to control the disease
• Topical corticosteroid creams are effective in treating lesions and preventing exacerbations
• Combination corticosteroid/ antibiotic creams are helpful, as treating the secondary infection is important
• Prolonged courses of oral antibiotics such as tetracycline may be useful
• A number of other oral medications (retinoid, cyclosporine, dapsone, and methotrexate) have been reported in single cases as partially effective
• Wet compresses to dry up oozing patches
• Botulinum toxin to reduce sweating or hyperhidrosis
• Phototherapy (ultraviolet light A and B) has also been used.
• Lasers have been reported to be useful in one study, vaporizing the affected skin.
• In severe cases surgery can be performed to remove the affected skin. Skin grafts are usually necessary to repair the wounds.