University of Washington School of Medicine, Seattle, WA, USA.
Division of Immunology, Department of Pediatrics, University of Washington School of Medicine, Seattle Children's Hospital, Seattle, WA, USA.
Clin Immunol. 2020 Feb;211:108330. doi: 10.1016/j.clim.2019.108330. Epub 2019 Dec 30.
Primary immunodeficiency Disorders (PIDD) are a varied group of heritable disorders characterized by defects in components of the innate and/or adaptive arms of the immune system. Although diagnosing these disorders is often challenging, the skin is a readily accessible and easily assessable organ that may provide clues to a diagnosis of PIDD. Specifically, many immunodeficiencies are associated with characteristic cutaneous eruptions that, based on their morphology, distribution and symptomatology, may suggest a specific underlying diagnosis. This review will discuss an approach to identifying and managing PIDDs that typically present with eczematous dermatitis.
原发性免疫缺陷病（PIDD）是一组遗传性疾病，其特征为固有和/或适应性免疫系统成分的缺陷。尽管诊断这些疾病通常具有挑战性，但皮肤是一种易于接近和评估的器官，它可能为 PIDD 的诊断提供线索。具体来说，许多免疫缺陷与特征性的皮肤疹有关，根据其形态、分布和症状，可能提示特定的潜在诊断。这篇综述将讨论一种识别和管理通常以湿疹性皮炎为表现的 PIDD 的方法。
