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Paraneoplastic pemphigus in association with Castleman's disease
O V Nikolskaia1, C H Nousari, G J Anhalt
1Department of Dermatology, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Ross Building, Suite 771, Baltimore, MD 21205, U.S.A.
The British Journal of Dermatology
|December 17, 2003
Summary
Paraneoplastic pemphigus (PNP) associated with Castleman's disease causes severe oral and skin lesions. Autoantibodies against plakin proteins are key diagnostic markers, with lung damage leading to fatal respiratory failure in most cases.
Area of Science:
- Immunodermatology
- Autoimmune diseases
- Oncology
Background:
- Paraneoplastic pemphigus (PNP) is a rare autoimmune mucocutaneous disease.
- It is strongly associated with lymphoproliferative neoplasms.
- Castleman's disease is a rare tumor frequently linked to PNP.
Purpose of the Study:
- To analyze clinical, immunopathological, and histopathological findings in patients with PNP and Castleman's disease.
- To identify diagnostic markers for this rare condition.
- To understand the disease's clinical course and outcomes.
Main Methods:
- Indirect immunofluorescence (IF) and immunoprecipitation (IP) for plakin autoantibodies.
- Immunoblotting for plectin autoantibodies.
- Enzyme-linked immunosorbent assay (ELISA) for desmoglein (Dsg)1 and Dsg3 autoantibodies.
Main Results:
- All patients presented with severe oral mucositis; 19/28 had lichenoid cutaneous lesions.
- Pulmonary destruction (bronchiolitis obliterans) occurred in 26 patients, leading to fatal respiratory failure in 22.
- Autoantibodies against desmoplakin I, envoplakin, periplakin, and plectin were detected in all or most patients.
Conclusions:
- PNP with Castleman's disease is characterized by severe oral and skin lesions.
- Serum autoantibodies against plakin proteins are the most reliable diagnostic markers.
- Pulmonary injury and respiratory failure are the primary causes of mortality.