Cutaneous multicentric Castleman's disease mimicking IgG4-related disease

Mai Takeuchi, Yasuharu Sato, Katsuyoshi Takata, Keita Kobayashi, Kyotaro Ohno, Noriko Iwaki, Yorihisa Orita, Tadashi Yoshino

Research output: Contribution to journalArticlepeer-review

26 Citations (Scopus)

Abstract

Castleman's disease, an uncommon lymphoproliferative disorder, can be difficult to differentiate from immunoglobulin (Ig) G4-related disease. The latter is typically characterized by elevated serum IgG4 levels and abundant IgG4-positive cells. However, multicentric Castleman's disease can also have elevated serum IgG4 levels and even fulfill the histological diagnostic criteria for IgG4-related disease. We present a case of cutaneous multicentric Castleman's disease mimicking IgG4-related disease. A 55-year-old Japanese woman developed erythematous and brown plaques on her back. Skin biopsy revealed regressive follicles with interfollicular plasmacytosis, and many plasma cells were positive for IgG4 (mean 263.67. ± 79.19, range 214-355 per high power field). The IgG4-/IgG-positive cell ratios were 35.6%, 36.2%, and 48.4%, respectively, with an average of 40.6%, thus fulfilling the histological diagnostic criteria for IgG4-related disease. Furthermore, serum IgG4 level was significantly elevated (1490. mg/dl; normal range: 4.8-105. mg/dl). However, laboratory findings of anemia, hypoalbuminemia, polyclonal gammaglobulinemia, high C-reactive protein level, and elevated serum interleukin-6 level were consistent with hyper-IL-6 syndrome. Hence, the diagnosis of cutaneous multicentric Castleman's disease was made. In conclusion, IgG4-related disease cannot be differentiated from hyper-IL-6 syndromes on histology alone. Instead, laboratory analyses are necessary to distinguish between the two diseases.

Original languageEnglish
Pages (from-to)746-749
Number of pages4
JournalPathology Research and Practice
Volume208
Issue number12
DOIs
Publication statusPublished - Dec 15 2012

Keywords

  • Castleman's disease
  • Cutaneous involvement
  • IL-6
  • IgG4-related disease

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Cell Biology

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