Dhouib Naouel Guirat, Ben Khaled Monia, Ouederni Monia, Ben-Mustapha Imen, Kouki Ridha, Besbes Habib, Barbouche Mohamed Ridha, Mellouli Fethi, Bejaoui Mohamed
Pediatric Immunohematology Department, Bone Marrow Transplantation Center Tunis, Tunisia.
Laboratory of Transmission, Control and Immunobiology of Infections (LR11IPT02), Institut Pasteur de Tunis, 1002 Tunis, Belvédère, Tunisia.
Mediterr J Hematol Infect Dis. 2018 Nov 1;10(1):e2018065. doi: 10.4084/MJHID.2018.065. eCollection 2018.
Skin manifestations are frequent among patients with primary immunodeficiency diseases (PIDs). Their prevalence varies according to the type of immunodeficiency. This review provides the reader with an up-to-date summary of the common dermatologic manifestations of PIDs among Tunisian children. We conducted a prospective study on two hundred and ninety children with immune deficiency. Demographic details (including age, sex, and consanguinity) with personal and family history were recorded. Special attention was paid to cutaneous manifestations. Dermatological involvements were grouped according to the etiology of their most prominent sign. Cutaneous manifestations were found in 164 patients (56.5%). They revealed the diagnosis of PIDs in 71 patients (24.5 %). The mean age at presentation was 21 months. Overall the most prominent cutaneous alterations were infectious. They accounted for 106 cases (36.55%). The most prevalent causes of cutaneous infections were bacterial: 93 cases (32.06%). Immuno-allergic skin diseases were among the common findings in our study. These include eczematous dermatitis found in 62 cases (21.38%). Malignancy related PIDs was seen in a boy with Wiskott Aldrich syndrome. He developed Kaposi's sarcoma at the age of 14 months. Cutaneous changes are common among children with PIDs. In pediatric patients with failure to thrive, chronic refractory systemic manifestations often present in other family members, recurrent cutaneous infections unresponsive to adequate therapy, atypical forms of eczematous dermatitis or unusual features should arouse the suspicion of PIDs and prompt specialized immunologic consultation should be made.
皮肤表现在原发性免疫缺陷病（PID）患者中很常见。其患病率因免疫缺陷类型而异。本综述为读者提供了突尼斯儿童中PID常见皮肤表现的最新总结。我们对290名免疫缺陷儿童进行了一项前瞻性研究。记录了人口统计学细节（包括年龄、性别和近亲结婚情况）以及个人和家族病史。特别关注皮肤表现。根据最突出体征的病因对皮肤受累情况进行分类。164例患者（56.5%）出现皮肤表现。其中71例患者（24.5%）通过皮肤表现确诊为PID。出现皮肤表现的平均年龄为21个月。总体而言，最突出的皮肤改变是感染性的。有106例（36.55%）。皮肤感染最常见的原因是细菌感染：93例（32.06%）。免疫过敏性皮肤病是我们研究中的常见发现。其中包括62例（21.38%）的湿疹性皮炎。与恶性肿瘤相关的PID见于一名患有维斯科特-奥尔德里奇综合征的男孩。他在14个月大时患上了卡波西肉瘤。皮肤改变在PID患儿中很常见。对于生长发育迟缓、其他家庭成员常出现慢性难治性全身表现、对充分治疗无反应的复发性皮肤感染、非典型形式的湿疹性皮炎或不寻常特征的儿科患者，应怀疑患有PID，并应及时进行专业的免疫学咨询。
