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首页> 外文期刊>Reumatologia >A child with subcorneal pustular dermatosis responded to IVIG treatment (Sneddon-Wilkinson disease)
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A child with subcorneal pustular dermatosis responded to IVIG treatment (Sneddon-Wilkinson disease)

机译：患角膜下脓疱性皮肤病的儿童对IVIG治疗有反应（Sneddon-Wilkinson病）

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Subcorneal pustular dermatosis (SPD) is a rare, chronic, recurrent dermatosis characterised by sterile pustules. It develops mainly in middle-aged or elder women, but is also rarely seen in children. The exact aetiology of the disease is unknown. In literature, cases associated with IgA gammopathy have been reported. In this article; we report a case of a five-year-old girl who was diagnosed as SPD by clinical features, histopathological characteristics, and direct immunofluorescence analysis results. IgA was high, and IgG-IgM and CD19+ B cell were low. We noticed that during IVIG treatment for immunodeficiency, dermatological symptoms were recovered rapidly. Clinical profile of SPD and its association with systemic diseases may provide early detection of immune dysfunction.
机译：角膜下脓疱性皮肤病（SPD）是一种罕见的，慢性，复发性皮肤病，其特征是无菌脓疱。它主要在中年或老年妇女中发展，但在儿童中也很少见。该病的确切病因尚不清楚。在文献中，已经报道了与IgA性丙种球蛋白病相关的病例。在这篇文章中;我们报告了一例5岁女孩，该女孩通过临床特征，组织病理学特征和直接的免疫荧光分析结果被诊断为SPD。 IgA高，而IgG-IgM和CD19 + B细胞低。我们注意到在IVIG免疫缺陷治疗期间，皮肤病症状迅速恢复。 SPD的临床概况及其与全身性疾病的关系可能会提供对免疫功能异常的早期检测。

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