Paraneoplastic Pemphigus and Bronchiolitis Obliterans in a Patient with Splenic B-cell Lymphoma.
J Formos Med Assoc
; 106(9): 768-73, 2007 Sep.
Article
em En
| MEDLINE
| ID: mdl-17908666
ABSTRACT
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndromes Paraneoplásicas
/
Bronquiolite
/
Linfoma de Células B
/
Pênfigo
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
J Formos Med Assoc
Assunto da revista:
MEDICINA
Ano de publicação:
2007
Tipo de documento:
Article
País de afiliação:
Taiwan