Paraneoplastic Pemphigus and Bronchiolitis Obliterans in a Patient with Splenic B-cell Lymphoma.
Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome, is a rare disorder associated with underlying neoplasia. The common underlying neoplasms include non-Hodgkins lymphoma, chronic lymphocytic leukemia, and Castlemans 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.
Wang
Shiou-Han
SH
Department of Dermatology, National Taiwan University Hospital, Taipei, Taiwan.
Chu
Chia-Yu
CY
Chen
Hsuan-Hsiang
HH
Chang
Yih-Leong
YL
Chen
Kuan-Yu
KY
Chiu
Hsien-Ching
HC
D000328:Adult; D001988:Bronchiolitis / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D006801:Humans; D016393:Lymphoma, B-Cell / Q000175:diagnosis*; D010257:Paraneoplastic Syndromes / Q000175:diagnosis*; D010392:Pemphigus / Q000175:diagnosis / Q000209:etiology*