[作为先天性免疫错误指征的相关皮肤表现]。

Dermatologie (Heidelberg, Germany) Pub Date : 2024-07-01 Epub Date: 2024-06-10 DOI:10.1007/s00105-024-05331-w
Leonie Frommherz, Larissa Akçetin, Fabian Hauck, Kathrin Giehl
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引用次数: 0

摘要

先天性免疫错误(IEI)可影响免疫系统的不同部分,尤其表现为病理性感染易感性和免疫失调。先天性免疫缺陷的皮肤表现可暗示潜在的免疫缺陷以及感染和炎症倾向。这些表现可表现为反复发作的湿疹、红斑、脓肿和脱发,且对治疗反应不佳。皮肤表现可以是某些 IEI 的特异性表现,也可以是非特异性表现。皮肤表现与临床病程和严重程度一起,可以提示诊断。早期准确的识别、诊断和治疗对于优化患者预后至关重要。诊断可通过详细的病史、临床检查和免疫学诊断来确定。免疫学专家和皮肤病专家之间的合作对于全面护理和提高生活质量至关重要。
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[Relevant cutaneous manifestations as indications for inborn errors of immunity].

Inborn errors of immunity (IEI) can affect different parts of the immune system and manifest especially through pathological infection susceptibility and immune dysregulation. Cutaneous manifestations of IEI can hint at the underlying immunodeficiency and the tendency for infection and inflammation. These manifestations can present as recurring eczema, erythema, abscesses, and hair loss with poor response to therapy. Cutaneous manifestations can be specific for certain IEI, or rather unspecific. Together with clinical course and severity, they can indicate the diagnosis. Early and accurate recognition, diagnosis, and treatment are crucial for optimizing patient outcomes. The diagnosis can be determined through a detailed patient history, clinical examination, and immunological diagnostics. Collaboration between immunologists and dermatologists is vital for comprehensive care and improvement of life quality.

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