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 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.
Lane
Joshua E
JE
Section of Dermatology, Department of Medicine, Medical College of Georgia, 1004 Chafee Ave, Augusta, GA 30912, USA.
Woody
Carol
C
Davis
Loretta S
LS
Guill
Margaret F
MF
Jerath
Rita S
RS
D001989:Bronchiolitis Obliterans / Q000209:etiology; D005871:Castleman Disease / Q000150:complications / Q000175:diagnosis*; D002648:Child; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D010257:Paraneoplastic Syndromes / Q000175:diagnosis*; D010392:Pemphigus / Q000175:diagnosis*; D012186:Retroperitoneal Neoplasms / Q000150:complications / Q000175:diagnosis*