PDF(Pubmed)
Abstract：
Dermatophytic pseudomycetoma is a rare invasive infection, involving both immunocompetent and immunocompromised individuals. Since the discovery of inherited immune disorders such as the impairment of CARD9 gene, extended dermatophyte infections are mostly ascribed to any of these host factors. This study is to present and explore the potential causes in a fatal dermatophytic pseudomycetoma patient. We present a chronic and deep pseudomycetoma caused by the common dermatophyte Microsporum canis which ultimately led to the death of the patient. Mycological examination, genetic studies and host immune responses against fungi were performed to explore the potential factors. The patient had decreased lymphocyte counts with significantly reduced CD4+ T cells, although all currently known genetic parameters proved to be normal. Through functional studies, we demonstrated that peripheral blood mononuclear cells from the patient showed severe impairment of adaptive cytokine production upon fungus-specific stimulation, whereas innate immune responses were partially defective. This is, to our knowledge, the first report of fatal dermatophytic pseudomycetoma in a patient with non-HIV CD4 lymphocytopenia, which highlights the importance of screening for immune deficiencies in patients with deep dermatophytosis.
摘要：
皮肤科假单胞菌瘤是一种罕见的侵袭性感染，涉及免疫能力和免疫功能低下的个体。自从发现遗传性免疫疾病如CARD9基因受损以来，扩展的皮肤癣菌感染主要归因于任何这些宿主因素。这项研究旨在介绍和探索致命的皮肤癣菌类假性肌瘤患者的潜在原因。我们提出了一种由常见的皮肤癣菌小孢子虫引起的慢性和深层假性细菌瘤，最终导致患者死亡。真菌学检查,进行了遗传研究和宿主对真菌的免疫反应，以探索潜在的因素。患者淋巴细胞计数减少，CD4+T细胞显著减少，尽管目前所有已知的遗传参数都被证明是正常的。通过功能研究，我们证明，患者的外周血单核细胞在真菌特异性刺激后表现出适应性细胞因子产生的严重损害，而先天免疫反应是部分缺陷。这是,根据我们的知识，首次报告了非HIVCD4淋巴细胞减少症患者的致命性皮肤真菌假性肌瘤，这突出了筛查深部皮肤癣菌病患者免疫缺陷的重要性。
