Clinical, Cosmetic and Investigational Dermatology (Nov 2022)
Coexistence of Discoid Lupus Erythematosus and Paraneoplastic Pemphigus: A Case Report and Literature Review
Abstract
Wasin Saowaluksakul,1 Chutima Seree-aphinan,1 Suthinee Rutnin,1 Kochawan Boonyawat,2 Kumutnart Chanprapaph1 1Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; 2Division of Hematology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, ThailandCorrespondence: Kumutnart Chanprapaph, Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Ratchatewi, Bangkok, 10400, Thailand, Tel +66 2 201 1141, Fax +66 2 201 1211, Email [email protected]: Pemphigus and lupus erythematosus are both B-cell-mediated autoimmune diseases, dependent on autoreactive CD4+ T lymphocytes to modulate autoimmune B-cell response. Many forms of pemphigus have been reported to occur in association with systemic lupus erythematosus (SLE) as well as other autoimmune diseases. However, it remains unclear whether this association occurs because of a shared immunopathogenesis or the coexistence may be coincidental. We hereby present a case report of discoid lupus erythematosus and paraneoplastic pemphigus associated with marginal zone lymphoma in a 54-year-old Thai man who had persistent oral erosions for 1 year together with generalized polymorphic cutaneous eruptions for 2 months. Simultaneous occurrence of paraneoplastic pemphigus and discoid lupus erythematosus without SLE has never been reported in the same individual. Hydroxychloroquine, immunosuppressive agents including prednisolone and azathioprine together with chemotherapy were given to treat these conditions.Keywords: paraneoplastic pemphigus, discoid lupus erythematosus, cutaneous lupus erythematosus, autoimmune bullous disease, non-Hodgkin lymphoma, marginal zone lymphoma