Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Overlap of IgG4-related Disease and Multicentric Castleman's Disease in a Patient with Skin Lesions
Hiroki MochizukiManako KatoTakakazu HiguchiRyosuke KoyamadaSatoru AraiSadamu OkadaHikaru Eto
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JOURNAL OPEN ACCESS

2017 Volume 56 Issue 9 Pages 1095-1099

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Abstract

A 59-year-old man presented with multiple dark red erythemas with induration, anemia, and polyclonal hypergammaglobulinemia. A skin biopsy revealed the infiltration of lymphocytes and plasma cells and he was initially diagnosed with multicentric Castleman's disease (MCD). Glucocorticoid treatment was only partially effective. Four years later, the patient's bilateral lacrimal glands gradually became enlarged and a biopsy revealed dense lymphocyte and plasma cell infiltration with an IgG4+/IgG+ plasma cell ratio of 70%. The patient was diagnosed with IgG4-related disease (RD). Rituximab only had a slight effect. This case demonstrates that overlapping features of IgG4-RD and MCD may present in a single patient, which suggests a shared pathogenesis.

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© 2017 by The Japanese Society of Internal Medicine
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