IgG4相关性皮肤病

IgG4-related skin disease

报告IgG4相关性皮肤病(IgG4-RSD)1例。患者男,25岁。躯干、面颈部瘙痒性红色至褐色丘疹、斑块及结节6年。皮损组织病理示真皮内胶原纤维增生,细血管周围小团块状或结节状淋巴细胞、浆细胞浸润。免疫组化示浆细胞表达CD38、CD138、CD19、κ-轻链、λ-轻链、IgG、IgG4均阳性,IgG4+/IgG+浆细胞比值>40%,每高倍视野下IgG4+浆细胞>10个。该文对IgG4-RSD的诊断、鉴别诊断和治疗进行讨论。

A case of IgG4-related skin disease is reported. A 25-year-old man presented with pruritic erythemas, brown papules, plaques and nodules on the face, neck and trunk for 6 years. Histopathological examination indicated that collagen fiber hyperplasia, perivaseular nodular or massive infiltrations of lymphocytes and plasmaeytes in the dermis. Immunohistochemistry revealed that plasma cells were positive for CD38, CD138, CD19, K, k, IgG and IgG4, the ratio of IgG4＋/IgG＋ plasma cells was more than 40%, the number of IgG4＋ plasma cells/HPF was over 10. The diagnosis of IgG4-related skin disease was made.