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Exacerbation of paraneoplastic pemphigus by cyclophosphamide treatment: detection of novel autoantigens and bronchial autoantibodies
- Source :
- British Journal of Dermatology. 150:1018-1024
- Publication Year :
- 2004
- Publisher :
- Oxford University Press (OUP), 2004.
-
Abstract
- A 48-year-old woman with a follicular, grade III, B-cell non-Hodgkin lymphoma developed clinical, immunopathological and histological features of paraneoplastic pemphigus. The skin symptoms flared after repeated cyclophosphamide infusions, and were preceded and accompanied by a progressive dyspnoea. Although the skin and oral mucosal disease went into remission with high-dose steroid and intravenous immunoglobulin therapy, the severe alveolitis led to death. Immunoblotting of human epidermal extracts showed that the patient's serum IgG reacted with the 210-kDa envoplakin, 190-kDa periplakin, as well as the recombinant protein of BP180 NC16a domain. IgG and IgA enzyme-linked immunosorbent assays for desmoglein 3 were positive, too. Indirect immunofluorescence studies on COS-7 cells transiently transfected with desmocollin 1-3 cDNAs showed that the patient's serum contained IgG and IgA antibodies to desmocollin 3 as well as IgG antibodies to desmocollin 2. Serum IgG and IgA strongly stained rat bronchial epithelium, corresponding to autoantibodies possibly involved in the pathomechanism of the severe lung disease. In this case, which was characterized by a mixed IgA/IgG antibody panel displaying known and unique antigenicity, the serious episodes of paraneoplastic pemphigus flared after cyclophosphamide treatment.
- Subjects :
- Pathology
medicine.medical_specialty
Envoplakin
Paraneoplastic Syndromes
Fluorescent Antibody Technique
Bronchi
Dermatology
Autoantigens
Fatal Outcome
Intravenous Immunoglobulin Therapy
medicine
Humans
education
Antineoplastic Agents, Alkylating
Cyclophosphamide
Periplakin
Autoantibodies
education.field_of_study
business.industry
Autoantibody
Middle Aged
medicine.disease
Pemphigus
Paraneoplastic pemphigus
Desmoglein 3
Immunology
Female
Drug Eruptions
Desmocollin
business
Subjects
Details
- ISSN :
- 13652133 and 00070963
- Volume :
- 150
- Database :
- OpenAIRE
- Journal :
- British Journal of Dermatology
- Accession number :
- edsair.doi.dedup.....f5476a3f5526bc968b5febc10df006b0