Benign Chronic Pemphigus (BCPM)

Synonyms: Familial benign chronic pemphigus, Hailey-Hailey disease.

This is a rare inherited autosomal dominant skin disorder, caused by a genetic mutation in the ATP2C1 gene located on chromosome 3.¹ Occasionally sporadic cases do occur. There is a defect in keratinocyte cohesion, first described in 1939 by brothers Hailey and Hailey (both dermatologists).²

May start in teenage years but most commonly presents in fourth and fifth decades. Vesicular or bullous rash often develops first in skin folds (axilla and groin),³ where malodourous erythematous plaques may develop.⁴ Trunk and neck can also be affected, with lesions provoked by friction, sun exposure, heat, and trauma.¹

Rash may be mistaken for pemphigus vulgaris, impetigo or fungal infection.

Skin biopsy may be required, although appearance and family history is usually enough.

• Supportive, avoid trigger factors (e.g. sunburn).
• Topical corticosteroids can help ±antibacterials.
• Prolonged courses of oral antibiotics (e.g. tetracycline) may be needed.
• Oral aciclovir may be useful if herpes virus infection is a problem.
• Calcipotriol cream has been used with success in some patients, as has phototherapy. Other drugs have been used such as retinoids, ciclosporin, dapsone, and methotrexate but no clinical trials have been performed.
• Lasers have been useful as reported in one study, vaporising the affected skin areas.⁵
• In severe cases, affected areas can be removed surgically, but skin grafts may be required to close the skin deficit.