TY - JOUR
T1 - Cutaneous multicentric Castleman's disease mimicking IgG4-related disease
AU - Takeuchi, Mai
AU - Sato, Yasuharu
AU - Takata, Katsuyoshi
AU - Kobayashi, Keita
AU - Ohno, Kyotaro
AU - Iwaki, Noriko
AU - Orita, Yorihisa
AU - Yoshino, Tadashi
N1 - Funding Information:
This work was supported by a Grant-in-Aid for Scientific Research (C) (no. 24591447) from the Japan Society for the Promotion of Science (JSPS) and “Research on Measures for Intractable Disease” Project: matching fund subsidy from Ministry of Health Labour and Welfare, Japan.
PY - 2012/12/15
Y1 - 2012/12/15
N2 - 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.
AB - 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.
KW - Castleman's disease
KW - Cutaneous involvement
KW - IL-6
KW - IgG4-related disease
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U2 - 10.1016/j.prp.2012.09.006
DO - 10.1016/j.prp.2012.09.006
M3 - Article
C2 - 23102767
AN - SCOPUS:84870300364
VL - 208
SP - 746
EP - 749
JO - Pathology Research and Practice
JF - Pathology Research and Practice
SN - 0344-0338
IS - 12
ER -