Hiremath N C, Madan Mohan N T, Srinivas C, Sangolli Prabhakar M, Srinivas K, Soumya N
Department of Dermatology, Dr. B.R. Ambedkar Medical College and Hospital, K.G. Halli, Bangalore - 560 045, Karnataka, India.
Indian J Dermatol. 2009;54(4):378-9. doi: 10.4103/0019-5154.57619.
Hyper IgE Syndrome (HIES) is a rare multi system genetic immunodeficiency disorder, with immunological and non-immunological features. Immunolgical features are 1) Recurrent cutaneous abscesses, 2) Atopic dermatitis like lesions, 3) Sino pulmonary infections, 4) Elevated serum IgE levels and 5) Abnormal neutrophil chemotaxis. Non immunological features include cranio facial and skeletal abnormalities. We are reporting a girl with classical features of HIES with umbilical hernia with her younger brother suffering from right sided inguinal hernia, as both herniae are hitherto unreported in patients with HIES.
高免疫球蛋白E综合征（HIES）是一种罕见的多系统遗传性免疫缺陷疾病，具有免疫和非免疫特征。免疫特征包括：1）复发性皮肤脓肿；2）特应性皮炎样病变；3）鼻窦肺部感染；4）血清IgE水平升高；5）中性粒细胞趋化异常。非免疫特征包括颅面部和骨骼异常。我们报告了一名具有HIES典型特征并伴有脐疝的女孩，其弟弟患有右侧腹股沟疝，因为这两种疝在HIES患者中均未见报道。
