Published online October 1, 2004
PEDIATRICS Vol. 114 No. 4 October 2004, pp. e513-e516 (doi:10.1542/peds.2004-0436)
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ELECTRONIC ARTICLE

Paraneoplastic Autoimmune Multiorgan Syndrome (Paraneoplastic Pemphigus) in a Child: Case Report and Review of the Literature

Joshua E. Lane, MD*, Carol Woody, MD*, Loretta S. Davis, MD*, Margaret F. Guill, MD{ddagger} and Rita S. Jerath, MD§

* Section of Dermatology, Department of Medicine
{ddagger} Section of Pulmonology, Department of Pediatrics
§ Section of Rheumatology, Department of Pediatrics, Medical College of Georgia, Augusta, Georgia

Paraneoplastic autoimmune multiorgan syndrome, also known as paraneoplastic pemphigus, has been observed only rarely among children. We describe a 10-year-old boy with typical clinical and histologic findings of paraneoplastic pemphigus associated with Castleman’s disease. His disease was refractory to resection of the tumor and aggressive combination immunosuppressive therapies. The patient died 1 year after presentation, as a result of complications of bronchiolitis obliterans. This case is unusual because of the young age of the patient.

Key Words: paraneoplastic pemphigus • paraneoplastic autoimmune multiorgan syndrome • child • Castleman’s tumor

Abbreviations: PAMS, paraneoplastic autoimmune multiorgan syndrome • CT, computed tomography • C3, complement component 3 • Ig, immunoglobulin

Accepted May 19, 2004.