Department of Dermatology, Osaka University Graduate School of Medicine, Osaka, Japan.
J Dermatol. 2010 Feb;37(2):171-4. doi: 10.1111/j.1346-8138.2009.00781.x.
Good's syndrome (GS) is a rare acquired combined T- and B-cell immunodeficiency accompanying thymoma. This report concerns a case of a 57-year-old man with GS manifesting intractable opportunistic infections and hyperkeratotic lichen planus. He had a past history of extended thymectomy for removal of thymoma. He consulted us about scaly and exudative intractable erythematous plaque on his right forearm. The histology was compatible with phlegmon coexisting with lichen planus. Laboratory examination results indicated hypogammaglobulinemia accompanied by complete absence of B cells, which is consistent with GS. Combined treatment with immunoglobulin replacement and administration of antibiotics and antifungal drugs was effective for the phlegmon and overlying fungal infection. The patient also presented with hyperkeratotic lichen planus on both knees and the right elbow, suggesting that intractable opportunistic infection and lichen planus may be associated with GS.
Good 综合征（GS）是一种罕见的获得性 T 细胞和 B 细胞联合免疫缺陷，伴有胸腺瘤。本报告涉及一例 57 岁男性 GS 患者，表现为难治性机会性感染和角化过度性扁平苔藓。他曾因胸腺瘤行扩大胸腺切除术。他因右前臂鳞屑性渗出性难治性红斑斑块来我院就诊。组织学符合伴有扁平苔藓的痈。实验室检查结果表明低丙种球蛋白血症伴有 B 细胞完全缺失，符合 GS 诊断。免疫球蛋白替代治疗联合抗生素和抗真菌药物治疗对痈和真菌感染有效。该患者的双侧膝关节和右侧肘部也出现角化过度性扁平苔藓，提示难治性机会性感染和扁平苔藓可能与 GS 有关。
