IgG4相关性皮肤病三例报道并文献复习

IgG4相关性疾病是一种多系统疾病，皮肤受累罕见，本文报道三例IgG4相关性皮肤病并复习相关文献。三例患者皮损表现为瘙痒性浸润性结节或斑块，分布于面颊、眼睑、耳周、颈部及胸部，同时伴有腮腺及泪腺相关症状，皮肤组织病理显示真皮到皮下脂肪层块状淋巴细胞及浆细胞浸润，IgG4+浆细胞浸润及血清IgG4水平显著升高。本病最有效的治疗方案是系统性应用糖皮质激素。

Three cases of IgG4-related skin disease and literature review

Three cases of IgG4-related skin disease were reported and he related literatures were reviewed. All the three cases in our study had uniform clinicopathological manifestation and high level serum IgG4. The clinical presentations were pruritic infiltrated nodules or plaques on the cheek, eyelid, periauricular region, neck and chest, together with related symptoms of lacrimal glands and parotid glands. Histopathology of the lesions revealed infiltration of lymphocytes and plasma cells in the dermis and subcutaneous tissue, including abundant IgG4-positive plasma cells. Glucocorticoid remain the most effective management for IgG4-related skin disease.