Skin manifestations in children with inborn errors of immunity in a tertiary care hospital in Iran

Zahra Salehi Shahrbabaki MD, Zahra Chavoshzadeh MD, Fahimeh Abdollahimajd MD, Samin sharafian MD, Mahnaz Jamee MD, Anastasia Bondarenko MD, Tolue Mahdavi MD
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Abstract

Objectives

Inborn errors of immunity (IEIs) are monogenic diseases of the immune system. Despite the increasing genetic advancements, the diagnosis of IEIs still lean on clinical diagnosis. Dermatological manifestations are observed in a large number of IEI patients and can lead to proper approach and prompt intervention.

Methods

This cross-sectional study was carried out between 2018 and 2020 on IEIs at a Children's tertiary care center in Tehran, Iran. Demographic details and age at onset of symptoms of IEI were recorded.

Results

Overall, 212 patients were included. Cutaneous findings were reported in 95 (44.8%) patients, and 61 of 95 (64.2%) reported skin lesions as the first clinical presentation. Skin infection (69, 72.6%) was the most frequent cutaneous manifestation, followed by eczematous rash (24, 25%).

Conclusions

Skin manifestations are a common feature in IEI patients and are readily recognizable by healthcare providers. This study tried to provide information on prognostic consequences.

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伊朗一家三级护理医院先天性免疫缺陷儿童的皮肤表现
先天性免疫失误是免疫系统的单基因疾病。尽管遗传技术不断进步,但IE的诊断仍然依赖于临床诊断。在大量IEI患者中观察到皮肤病表现,可以采取适当的方法并及时干预。
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CiteScore
0.60
自引率
10.00%
发文量
69
审稿时长
12 weeks
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