Achievement of the longest survival of paraneoplastic pemphigus with bronchiolitis obliterans associated with follicular lymphoma using R-CHOP chemotherapy

Paraneoplastic pemphigus (PNP) is a rare, fatal, paraneoplastic autoimmune mucocutaneous blistering disease, commonly associated with lymphoproliferative disorders, including malignant lymphomas. Lymphoproliferative disorders associated with PNP are sometimes associated with a serious lung complication, bronchiolitis obliterans (BO). Due to its rarity, guidelines for the management of PNP have not been established. Furthermore, most patients die within 1 year. Here we report the successful treatment of lymphoma-associated PNP and BO using R-CHOP chemotherapy. A 53-year-old Japanese man was admitted to our hospital for severe erosive stomatitis. Computed tomography and positron emission tomography showed multiple lymphadenopathies. He was diagnosed with follicular lymphoma (Ann Arbor stage IVA) and PNP-related BO. The patient underwent six cycles of R-CHOP and an additional cycle of rituximab. Both the erosive stomatitis and the obstructive lung disease persisted, but complete response of the follicular lymphoma was achieved. The patient survived 27 months after diagnosis. Although he died from progressive respiratory failure due to BO, we note that this patient achieved the longest survival of any reported case of PNP-related BO associated with a lymphoproliferative disorder. The present case suggests that intensive immunochemotherapy for underlying lymphoma may improve the prognosis in patients with PNP-related BO associated with lymphoma.