Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare immune deficiency with a broad clinical presentation. IPEX syndrome causes dysfunctional regulatory T cells, increasing the risk of autoimmune diseases. In this case report, we describe a 7-year-old boy with lymphocytic interstitial pneumonia and bullous pemphigoid who was recently diagnosed with IPEX syndrome.

中文翻译：

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免疫失调、多内分泌病、肠病、X-连锁综合征表现为淋巴细胞性间质性肺炎和难治性大疱性类天疱疮

免疫失调、多内分泌病、肠病、X 连锁 (IPEX) 综合征是一种罕见的免疫缺陷，临床表现广泛。IPEX 综合征会导致功能失调的调节性 T 细胞，增加自身免疫性疾病的风险。在本病例报告中，我们描述了一名 7 岁男孩，患有淋巴细胞性间质性肺炎和大疱性类天疱疮，他最近被诊断出患有 IPEX 综合征。