Glick Laura R, Wilson William Wyatt, Fletcher Michelle
Pritzker School of Medicine, University of Chicago, Chicago, Illinois, USA.
Department of Medicine, University of Chicago, Chicago, Illinois, USA.
BMJ Case Rep. 2019 Aug 20;12(8):e229999. doi: 10.1136/bcr-2019-229999.
Good's syndrome (GS) is a rare, adult-onset combined B cell and T cell immunodeficiency with an associated thymoma. These patients have an increased risk of bacterial, fungal, viral and opportunistic infections. This report describes a 75-year-old female patient who presented with a full body rash and an anterior mediastinal mass. She underwent a biopsy of her rash and mass, which revealed erythema multiforme and WHO Type A thymoma, respectively. During her hospitalisation, she was also found to have oropharyngeal candidiasis, methicillin-susceptible bacteraemia and herpes simplex virus type 2 (HSV-2) skin lesions. Based on the number of infections and severity of her rash, an immunocompromised state was suspected. Immunological testing revealed a B cell and T cell deficiency as well as low serum immunoglobulins. This combination of hypogammaglobulinaemia and thymoma led to a diagnosis of GS. While there have been many case reports of GS, this is the first report of the immunodeficiency presenting with erythema multiforme.
古德综合征（GS）是一种罕见的、成人起病的B细胞和T细胞联合免疫缺陷病，并伴有胸腺瘤。这些患者发生细菌、真菌、病毒和机会性感染的风险增加。本报告描述了一名75岁女性患者，她出现全身皮疹和前纵隔肿块。她接受了皮疹和肿块活检，结果分别显示为多形红斑和WHO A型胸腺瘤。在住院期间，还发现她患有口腔念珠菌病、甲氧西林敏感菌血症和2型单纯疱疹病毒（HSV-2）皮肤病变。基于感染次数和皮疹严重程度，怀疑存在免疫功能低下状态。免疫学检测显示B细胞和T细胞缺乏以及血清免疫球蛋白水平低下。低丙种球蛋白血症和胸腺瘤的这种组合导致了GS的诊断。虽然已有许多GS的病例报告，但这是首例以多形红斑为表现的免疫缺陷病例报告。
