Paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) in a child: Case report and review of the literature

被引：28

作者：

Lane, JE

Woody, C

Davis, LS

Guill, MF

Jerath, RS

机构：

[1] Med Coll Georgia, Dept Med, Dermatol Sect, Augusta, GA 30912 USA

[2] Med Coll Georgia, Dept Pediat, Sect Pulmonol, Augusta, GA 30912 USA

[3] Med Coll Georgia, Dept Pediat, Rheumatol Sect, Augusta, GA 30912 USA

来源：

PEDIATRICS | 2004年 / 114卷 / 04期

关键词：

paraneoplastic pemphigus; paraneoplastic autoimmune multiorgan syndrome; child; Castleman's tumor;

D O I：

10.1542/peds.2004-0436

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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.

引用

页码：E513 / E516

页数：4

共 29 条

[1] Antibodies against desmoglein 3 (Pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice [J].