Korean J Dermatol.  2018 Mar;56(3):197-201. 10.0000/kjd.2018.56.3.197.

IgG4-related Skin Disease: Experience with Two Cases

  • 1Department of Dermatology, College of Medicine, Chungnam National University, Daejeon, Korea. jhoon@cnu.ac.kr


IgG4-related disease (IgG4-RD) is a new disease entity characterized by elevated serum IgG4 and infiltration of IgG4+ plasma cells in tissue. IgG4-RD can involve various organs, and skin could also be affected. The manifestations of IgG4-related skin disease (IgG4-RSD) are not characteristic, however it usually presents with multiple erythematous nodules or plaques with itching sensation. We report two cases of IgG4-RSD. Histological studies of these cases revealed infiltration of numerous plasma cells and mononuclear cells in the entire dermis. Some plasma cells were strongly positive for IgG4 stain. IgG4-RSD is a treatable disease with systemic corticosteroids. Thus, clinicians should obtain a biopsy and identify IgG4-positive cells for an accurate diagnosis.


IgG4-related disease
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