| Summary: | Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome, is a rare disorder associated with underlying neoplasia. The common underlying neoplasms include non-Hodgkin's lymphoma, chronic lymphocytic leukemia, and Castleman's disease. Though B-cell lymphoma is the most common underlying malignancy, only one case associated with splenic B-cell lymphoma has been recognized. The prognosis of PNP is very poor, and PNP-associated bronchiolitis obliterans (BO) is not uncommon. Herein, we report a 44-year-old woman who initially presented with multiple oral ulcers, conjunctivitis, and numerous cutaneous blisters. Serial workup established the diagnosis of PNP and revealed an underlying splenic B-cell lymphoma. Although the mucocutaneous lesions gradually healed after splenectomy and chemotherapy, deteriorating respiratory function developed 7 months later with pathologically proven BO. She finally succumbed to respiratory failure 12 months after presentation despite intensive respiratory care.
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