López-Quintero William, Cleves Daniela, Gomez-Vasco Jose David, Pérez Paola, Patiño Jaime, Medina-Valencia Diego, Pachajoa Harry, Torres-Canchala Laura, Vidal Andres, Olaya Manuela
Fundación Valle del Lili, Servicio de Dermatología, Cali, Colombia.
Universidad Icesi, Facultad Ciencias de la Salud, Cali, Colombia.
World Allergy Organ J. 2021 Mar 1;14(3):100527. doi: 10.1016/j.waojou.2021.100527. eCollection 2021 Mar.
The current literature describes the characteristics of some skin manifestations in the context of primary immunodeficiency diseases (PIDs), also known as inborn errors of the immune system. However, there are hardly any data on the epidemiological trends of skin manifestations and PIDs in Latin America (LA). We aimed to describe the characteristics of patients with skin manifestations and the diagnosis of a PID treated at a tertiary hospital in Colombia.
This was a retrospective observational study. Data were taken from the institutional database of pediatric PIDs, which includes 306 patients under 18 years of age who attended a tertiary care center in Cali, Colombia for inpatient or outpatient services between December 2013 and December 2018. A trained third-year dermatology resident reviewed the electronic clinical records of all the patients in the database and double-checked patients who presented with cutaneous signs and symptoms.
A total of 83 patients out of the original 306 patients (27.1%) presented with some type of cutaneous manifestation. Of these patients, 56.6% had atopic dermatitis, 56.6% reported at least one episode of skin infection, and some of the patients had both of these manifestations. Infections were more frequent in the PID group of combined immunodeficiency associated with well-defined syndromes and atopic dermatitis in the group of antibody deficiencies.
It is important to recognize dermatological clinical characteristics in patients with PIDs. More studies are necessary to establish recommendations regarding the approach of diagnosis and management of these patients.
当前文献描述了原发性免疫缺陷病（PIDs）（也称为免疫系统先天性疾病）背景下一些皮肤表现的特征。然而，关于拉丁美洲（LA）皮肤表现和原发性免疫缺陷病的流行病学趋势的数据几乎没有。我们旨在描述在哥伦比亚一家三级医院接受治疗的有皮肤表现的患者特征以及原发性免疫缺陷病的诊断情况。
这是一项回顾性观察研究。数据取自儿科原发性免疫缺陷病的机构数据库，该数据库包括2013年12月至2018年12月期间在哥伦比亚卡利的一家三级护理中心接受住院或门诊服务的306名18岁以下患者。一名经过培训的三年级皮肤科住院医师查阅了数据库中所有患者的电子临床记录，并对出现皮肤体征和症状的患者进行了复查。
在最初的306名患者中，共有83名患者（27.1%）出现了某种类型的皮肤表现。在这些患者中，56.6%患有特应性皮炎，56.6%报告至少有一次皮肤感染发作，一些患者同时有这两种表现。在与明确综合征相关的联合免疫缺陷的原发性免疫缺陷病组中感染更为常见，而在抗体缺陷组中特应性皮炎更为常见。
认识原发性免疫缺陷病患者的皮肤病临床特征很重要。需要更多研究来制定关于这些患者诊断和管理方法的建议。
