Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disease with circulating antibodies that bind the cell surface of the epidermis and other non-stratifying epithelia, and immunoprecipitate a complex of four or five proteins (250 kDa, 230 kDa, 210 kDa, 190 kDa and occasionally 170 kDa). Combinations of immunosuppressive agents are usually required to obtain even partial control of the skin lesions. Mucous membrane lesions are refractory to treatment. We describe a patient with PNP whose skin and oral lesions are quiescent following treatment with oral mycophenolate mofetil.
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