Shenoy Adele, Mohandas Nirupa, Gottlieb Alice
New York Medical College, School of Medicine, Valhalla, NY.
Dermatol Online J. 2019 Jun 15;25(6):13030/qt9w91m8dz.
Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory condition characterized by IgG4+ plasma cell infiltration of the skin and other organs. Cutaneous forms of the disease may be under recognized owing to poorly defined diagnostic criteria and relatively recent recognition in the literature. The aim of this review is to describe the clinical, histological, and serological presentations of cutaneous IgG4-RD, and to provide an overview of its systemic manifestations for dermatologists. Cases of cutaneous IgG4-RD identified in the literature review were compared to control cases. Clinically, plaque morphology and systemic involvement of the orbit, submandibular gland, lacrimal gland, and parotid gland were associated with a diagnosis of cutaneous IgG4-RD. Histologically, lymphoplasmacytic infiltrate and percentage of IgG4+ plasma cells/IgG+ plasma cells > 40% were associated with the diagnosis. Serologically, neither elevated serum IgG4 nor IgE concentrations were associated with the diagnosis. Dermatologists should consider IgG4-RD as part of the differential diagnosis for nodules, papules, and plaques with an IgG4+ plasma cell infiltrate, especially in middle-aged and elderly males with systemic manifestations of the disease. Diagnosis requires thorough investigation of both cutaneous and systemic clinical and histological presentations.
免疫球蛋白G4相关疾病（IgG4-RD）是一种慢性炎症性疾病，其特征为皮肤和其他器官出现IgG4 +浆细胞浸润。由于诊断标准不明确以及在文献中相对较新的认识，该疾病的皮肤表现形式可能未得到充分认识。本综述的目的是描述皮肤IgG4-RD的临床、组织学和血清学表现，并为皮肤科医生提供其全身表现的概述。将文献综述中确定的皮肤IgG4-RD病例与对照病例进行比较。临床上，斑块形态以及眼眶、颌下腺、泪腺和腮腺的全身受累与皮肤IgG4-RD的诊断相关。组织学上，淋巴浆细胞浸润以及IgG4 +浆细胞/IgG +浆细胞百分比> 40%与诊断相关。血清学上，血清IgG4和IgE浓度升高均与诊断无关。皮肤科医生应将IgG4-RD视为具有IgG4 +浆细胞浸润的结节、丘疹和斑块鉴别诊断的一部分，尤其是在患有该疾病全身表现的中老年男性中。诊断需要对皮肤和全身的临床及组织学表现进行全面调查。
