Wang S.-H.CHIA-YU CHUChen H.-H.YIH-LEONG CHANGKUAN-YU CHENHSIEN-CHING CHIU2021-04-292021-04-2920070929-6646https://www.scopus.com/inward/record.uri?eid=2-s2.0-35348848576&doi=10.1016%2fS0929-6646%2808%2960038-8&partnerID=40&md5=aac86f13d56d23db8c6ddc71abfd6545https://scholars.lib.ntu.edu.tw/handle/123456789/558248Paraneoplastic 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. ?2007 Elsevier & Formosan Medical Association.[SDGs]SDG3aminophylline; CD20 antibody; complement component C1q; fibrinogen; hydrocortisone; immunoglobulin A; immunoglobulin G; immunoglobulin M; ipratropium bromide; prednisolone; terbutaline sulfate; vincristine; adult; article; asthma; atelectasis; B cell lymphoma; bronchiolitis obliterans; case report; clinical feature; computer assisted surgery; computer assisted tomography; fatality; female; histopathology; human; human tissue; immunofluorescence; intubation; lung collapse; lung function test; lung insufficiency; multiple cycle treatment; paraneoplastic syndrome; pemphigus; pulmonary rehabilitation; respiratory distress; resuscitation; skin biopsy; spleen tumor; splenectomy; thoracoscopy; thorax radiographyjournal article10.1016/S0929-6646(08)60038-8179086662-s2.0-35348848576