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

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 refra...

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Bibliographic Details
Published in:Pediatrics (Evanston) 2004-10, Vol.114 (4), p.e513-e516
Main Authors: Lane, Joshua E, Woody, Carol, Davis, Loretta S, Guill, Margaret F, Jerath, Rita S
Format: Article
Language:English
Subjects:
Bronchiolitis Obliterans - etiology
Castleman Disease - complications
Castleman Disease - diagnosis
Child
Fatal Outcome
Humans
Male
Paraneoplastic Syndromes - diagnosis
Pemphigus - diagnosis
Retroperitoneal Neoplasms - complications
Retroperitoneal Neoplasms - diagnosis
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Summary: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.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.2004-0436