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Multicentric Castleman's disease associated with inherited epidermolysis bullosa

Yoshio Kawakami1, Akiko Nishibu, Satoshi Kikuchi

  • 1Department of Dermatology, Fukushima Medical University School of Medicine, Fukushima, Japan.

The Journal of Dermatology
|October 28, 2003
PubMed

Multicentric Castleman's disease (MCD) is a rare disorder characterized by fever, polyclonal hypergammaglobulinemia, and generalized lymphadenopathy. It has three histological characteristics: a recognizable architecture, germinal center abnormalities, and plasmacytosis. Inherited epidermolysis bullosa (EB) is also a rare disorder caused by a genetic defect. We report a 43-year-old patient with dystrophic EB, non-Hallopeau-Siemens recessive type or dominant type, displaying clinicopathologic features of MCD. In addition, his serum interleukin-6, which is thought to be responsible for the clinical symptoms in MCD, was elevated.

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