Remission of Refractory Benign Familial Chronic Pemphigus (Hailey-Hailey Disease) with the Addition of Systemic Cyclosporine

Background: Benign chronic familial pemphigus (BFCP) is an autosomal dominant dermatosis characterized by flares of painful and often debilitating blistering lesions in high friction areas of the body such as the groin, axillae, lateral neck, and intergluteal cleft. Limited knowledge of its pathophysiology has made treatment of BFCP a considerable challenge and efficacy with current first line therapies, topical corticosteroids and antibiotics, is variable. Case Report: We present a case of this disease in a 52 year old woman that has responded dramatically to the addition of oral cyclosporine to her existing regimen of oral acitretin, with significant improvement of skin lesions, mobility, and quality of life. Cyclosporine’s mechanism of action in BFCP is poorly understood, although it possibly acts through inhibition of proinflammatory cytokines in keratinocytes or modulation of intracellular calcium. BFCP, the use of cyclosporine for its treatment, and possible mechanisms of action of cyclosporine are reviewed. Contexte: La maladie de Hailey-Hailey (MHH) (pemphigus bénin chronique familial) est une dermatose autosomique, dominante, qui se caractérise par l’apparition, en poussée, de lésions vésicantes, douloureuses, et souvent débilitantes sur des parties du corps soumises a ̀ une grande friction, telles que les aines, les aisselles, les parties latérales du cou, et le sillon interfessier. Le manque de connaissances sur la physiopathologie de la MHH en rend le traitement très difficile, sans compter que l’efficacite ́ des traitements actuels de première intention comme les corticostéroı̈sdes topiques et les antibiotiques est variable