Successful treatment with oral mizoribine in refractory ocular manifestation of mucosal pemphigus vulgaris: A unique response to different immunosuppressive drugs.

Pemphigus vulgaris (PV) is an acquired autoimmune disease in which the disease characteristic antibodies are directed against the desmosomal transmembrane glycoprotein, desmoglein 3 (Dsg 3), resulting in flaccid blisters and erosions of skin and mucous membrane. Among various affected sites, ocular involvement may often persist or relapse even after remission of other mucocutaneous lesions, and also represent a higher morbidity. We describe such an example case of mucosal PV, whose oral and ocular manifestations were responded specifically to oral cyclosporine and mizoribine, respectively. To our knowledge, this is the first case of the site-specific efficacy of mizoribine in PV.