Wang Weijia, Kang Xiaojing, Ding Yuan, Mao Lidan, Dilinuer Abudureyimu, Li Wenzheng
Department of Dermatology and Venereology, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, People's Republic of China.
Xinjiang Clinical Research Center for Dermatologic Diseases, Urumqi, People's Republic of China.
Clin Cosmet Investig Dermatol. 2023 Aug 2;16:1997-2004. doi: 10.2147/CCID.S406199. eCollection 2023.
IgG4-related disease (IgG4-RD) is a rare fibroinflammatory disease that has a high tendency to misdiagnosis in clinics.
A 48-year-old man developed a rash with progressive itching 3 years ago after hormone therapy for an ocular "inflammatory pseudotumor". The disease condition of this patient involved multiple organs which involved the skin. The patient was misdiagnosed with other diseases during the period of hospitalization, leading to poor therapeutic effects and repeated skin lesions. The dermatopathological report indicated plasma cell proliferative disorder, with IgG4/IgG exceeding 40% and abnormally elevated serum IgG4 levels. After the patient was diagnosed with IgG4-RD, a series of treatments improved skin lesions, relieved other symptoms, and decreased serum IgG4 levels.
IgG4-RD is a highly misdiagnosed disease that deserves the attention of physicians. The patient we reported could be considered a representative case of IgG4-RD that presents with skin lesions. For patients with suspected IgG4-RD, serum IgG4 testing should be performed, and further imaging, serological tests, and pathology examinations are needed to exclude malignancy, infection, and autoimmune diseases.
IgG4相关性疾病（IgG4-RD）是一种罕见的纤维炎性疾病，在临床上极易误诊。
一名48岁男性在3年前因眼部“炎性假瘤”接受激素治疗后出现皮疹，并伴有进行性瘙痒。该患者的病情累及多个器官，包括皮肤。患者在住院期间被误诊为其他疾病，导致治疗效果不佳且皮肤病变反复出现。皮肤病理报告显示浆细胞增殖性疾病，IgG4/IgG超过40%，血清IgG4水平异常升高。患者被诊断为IgG4-RD后，一系列治疗改善了皮肤病变，缓解了其他症状，并降低了血清IgG4水平。
IgG4-RD是一种极易误诊的疾病，值得医生关注。我们报告的该患者可被视为IgG4-RD伴皮肤病变的典型病例。对于疑似IgG4-RD的患者，应进行血清IgG4检测，还需要进一步的影像学、血清学检查及病理检查以排除恶性肿瘤、感染及自身免疫性疾病。
