Cansunar Reyhan S, Özden Güzin, Cevirme Leyla, Dik Susamber, Erkoc Merve, Basir Hakan
Allergy and Immunology, Adana City Training and Research Hospital, Adana, TUR.
Allergy and Immunology, Adana City Training and research Hospital, Adana, TUR.
Cureus. 2025 Mar 3;17(3):e79976. doi: 10.7759/cureus.79976. eCollection 2025 Mar.
Hyper IgE syndrome (HIES) is a rare primary immunodeficiency characterized by chronic eczema, recurrent staphylococcal infections on the skin and pulmonary system, and high serum IgE concentrations. The first clinical sign of HIES is eczema, usually in early infancy. Severe atopic dermatitis (AD) may also mimic HIES with findings of eczema, high serum immunoglobulin E levels, and eosinophilia. Therefore, differential diagnosis may be difficult. Here, we present a case with eczematoid skin rashes, asthma, elevated serum IgE levels, and skin infections that started in infancy and were followed for nine years by the pediatric allergy and immunology clinic with the diagnosis of HIES, but in fact, had severe AD. Because the patient had no recurrent infections that would suggest immune deficiency during his clinical follow-up, other than the skin infections at the time of diagnosis. In addition, the patient had no non-immunological symptoms of hyper IgE syndrome.
高免疫球蛋白E综合征（HIES）是一种罕见的原发性免疫缺陷病，其特征为慢性湿疹、皮肤和肺部系统反复发生葡萄球菌感染以及血清免疫球蛋白E浓度升高。HIES的首个临床症状是湿疹，通常在婴儿早期出现。严重特应性皮炎（AD）也可能与HIES相似，表现为湿疹、血清免疫球蛋白E水平升高和嗜酸性粒细胞增多。因此，鉴别诊断可能较为困难。在此，我们报告一例病例，该患者自婴儿期起出现湿疹样皮疹、哮喘、血清免疫球蛋白E水平升高及皮肤感染，小儿过敏与免疫门诊对其进行了9年的随访，诊断为HIES，但实际上该患者患有严重的AD。因为该患者在临床随访期间，除了诊断时的皮肤感染外，没有提示免疫缺陷的反复感染。此外，该患者没有高免疫球蛋白E综合征的非免疫性症状。
