Multicentric Castleman's disease associated with inherited epidermolysis bullosa

J Dermatol. 2003 Sep;30(9):689-93. doi: 10.1111/j.1346-8138.2003.tb00459.x.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Asian People / genetics
  • Castleman Disease / blood
  • Castleman Disease / complications
  • Castleman Disease / diagnosis*
  • Castleman Disease / genetics
  • Castleman Disease / pathology
  • Diagnosis, Differential
  • Epidermolysis Bullosa / blood
  • Epidermolysis Bullosa / complications
  • Epidermolysis Bullosa / diagnosis*
  • Epidermolysis Bullosa / genetics
  • Epidermolysis Bullosa / pathology
  • Genetic Predisposition to Disease*
  • Humans
  • Interleukin-6 / blood
  • Japan
  • Male

Substances

  • Interleukin-6