Good's syndrome (hypogammaglobulinemia with thymoma) presenting intractable opportunistic infections and hyperkeratotic lichen planus
Good's syndrome (hypogammaglobulinemia with thymoma) presenting intractable opportunistic infections and hyperkeratotic lichen planus
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DOI：
10.1111/j.1346-8138.2009.00781.x
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发表时间：
2010-02-01
影响因子：
3.1
通讯作者：
Katayama, Ichiro
中科院分区：
文献类型：
--
作者：
Hanafusa, Takaaki;Umegaki, Noriko;Katayama, Ichiro
古德氏综合征（GS）是一种罕见的获得性T细胞和b细胞联合免疫缺陷伴胸腺瘤。本报告涉及一个57岁男性GS表现难治性机会性感染和角化性扁平苔藓。他曾为切除胸腺瘤而进行过扩大胸腺切除术。他向我们咨询了他右前臂的鳞状和渗出性顽固性红斑斑块。组织学符合痰伴扁平苔藓。实验室检查结果显示低γ -球蛋白血症伴B细胞完全缺失，与GS一致。免疫球蛋白替代及抗生素、抗真菌药物联合治疗对痰及复盖真菌感染有效。患者双膝和右肘出现角化性扁平苔藓，提示顽固性机会性感染和扁平苔藓可能与GS有关。
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.