Multicentric Castleman's Disease Associated with Inherited Epidermolysis Bullosa
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...
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Veröffentlicht in: | Journal of dermatology 2003-09, Vol.30 (9), p.689-693 |
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Format: | Artikel |
Sprache: | eng |
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Zusammenfassung: | 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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ISSN: | 0385-2407 1346-8138 |
DOI: | 10.1111/j.1346-8138.2003.tb00459.x |