Division of Allergy, Immunology, and Rheumatology, Department of Pediatrics, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY.
Division of Allergy, Immunology, and Rheumatology, Department of Pediatrics, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY.
J Allergy Clin Immunol Pract. 2019 Mar;7(3):788-798. doi: 10.1016/j.jaip.2018.11.026.
Primary immune deficiency diseases characteristically present with recurrent, severe, or unusual infections. These infections may often involve the skin, with mucocutaneous candidal infections seen in a variety of different primary immune deficiencies. Primary immune deficiencies may also present with noninfectious cutaneous complications, of which eczema is the most common. In a patient with suspected primary immune deficiency, the presence of eczema or candidal skin infections offers critical information about the underlying immune defect, either the presence of atopy or defect in the T17 pathway, respectively. These skin manifestations also are often early or heralding findings of the underlying immunologic disease. Therefore, awareness of associations between these skin findings and specific immune deficiencies may aide in the early detection and treatment of serious or life-threatening immunologic defects. This review specifically will focus on the primary immune deficiencies commonly associated with eczema or mucocutaneous candidiasis.
原发性免疫缺陷病的特征是反复出现严重或不常见的感染。这些感染通常涉及皮肤，各种原发性免疫缺陷病均可出现黏膜皮肤念珠菌感染。原发性免疫缺陷病也可出现非感染性皮肤并发症，其中湿疹最为常见。在疑似原发性免疫缺陷病患者中，湿疹或念珠菌皮肤感染的存在为潜在免疫缺陷提供了关键信息，分别提示特应性或 T17 途径缺陷的存在。这些皮肤表现通常也是潜在免疫性疾病的早期或先驱发现。因此，了解这些皮肤表现与特定免疫缺陷之间的关联可能有助于早期发现和治疗严重或危及生命的免疫缺陷。本综述将专门讨论与湿疹或黏膜皮肤念珠菌病相关的常见原发性免疫缺陷病。
