Paraneoplastic pemphigus (PNP) is a rare, lethal, paraneoplastic autoimmune mucocutaneous blistering disease commonly associated with lymphoproliferative disorders including malignant lymphomas. Lymphoproliferative disorders associated with PNP are sometimes associated with serious lung complications such as bronchiolitis obliterans (BO). Because of its rarity, guidelines have not been established for management of PNP. Furthermore, most patients die within 1 year. We report treatment of lymphomaassociated PNP and BO using CHOP chemotherapy with azathioprine. A 57-year-old Thai woman suffered from systemic blisters, severe erosive stomatitis, and abdominal distension. Computed tomography revealed multiple lymphadenopathies. She was diagnosed with follicular lymphoma grade 1 (Ann Arbor stage IVA) and PNP-related BO. The patient underwent four cycles of CHOP and azathioprine. The erosive stomatitis and systemic blisters resolved completely. The lymph nodes became smaller with a partial response of the underlying follicular lymphoma. However, the bronchiolitis obliterans persisted. The patient achieved 15 months of survival. The present case suggests that intensive chemotherapy with immunosuppressive improves the prognosis of patients with PNP-related BO associated with lymphoma.

Keywords: Follicular lymphoma, Paraneoplastic pemphigus, Bronchiolitis obliterans