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遗传性皮肤病和原发性免疫缺陷的皮肤表现。

Cutaneous manifestations of genodermatoses and primary immunodeficiency.

作者信息

Lewis Daniel J, Wu Julie H, Boyd McKenna, Duvic Madeleine, Feldman Steven R

机构信息

Center for Dermatology Research, Wake Forest University School of Medicine, Winston-Salem, NC Departments of Pathology and Public Health Sciences, Wake Forest University School of Medicine, Winston-Salem, NC.

出版信息

Dermatol Online J. 2019 Jun 15;25(6):13030/qt1gj1n07j.

Abstract

Immunodeficiency is most commonly related to inherited syndromes, infections, chemotherapy, or aging. As the population of individuals with immunodeficiency continues to grow, physicians are confronted with the task of diagnosing dermatologic disease in this population. Cutaneous involvement in immunodeficiency disorders serves as a useful tool that aids diagnosis and provides prognostic value. Given that cutaneous manifestations often herald an underlying immunodeficiency disorder, understanding the complexities of these diseases is important for appropriate clinical management. Although certain diseases may present with stereotypical cutaneous lesions, others may involve more variable lesions that require specialized consultation for diagnosis and treatment recommendations. In this review, we discuss a number of cutaneous findings associated with primary immunodeficiencies. Awareness of these cutaneous associations may aid in the early detection and prompt treatment of these potentially serious immunologic disorders.

摘要

免疫缺陷最常与遗传性综合征、感染、化疗或衰老相关。随着免疫缺陷个体数量的持续增加,医生面临着在这一人群中诊断皮肤病的任务。免疫缺陷疾病中的皮肤受累是辅助诊断并具有预后价值的有用工具。鉴于皮肤表现常常预示着潜在的免疫缺陷疾病,了解这些疾病的复杂性对于恰当的临床管理很重要。虽然某些疾病可能表现为典型的皮肤损害,但其他疾病可能涉及更多可变的损害,需要专业会诊以提供诊断和治疗建议。在本综述中,我们讨论了一些与原发性免疫缺陷相关的皮肤表现。了解这些皮肤关联可能有助于早期发现并及时治疗这些潜在的严重免疫性疾病。

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