Ghaffari Javad, Abedian-Kenari Saeed, Ghasemi Maryam, Gohardehi Farzad
Department of Pediatrics, Mazandaran University of Medical Sciences, Sari, Iran.
Caspian J Intern Med. 2013 Summer;4(3):735-8.
Hyper IgE syndrome (HIES) is a rare primary immune deficiency, described as Job(`)s syndrome characterized by increased serum levels of IgE, eczema, recurrent cutaneous and pulmonary infections. In this paper, we presented a case of Hyper IgE syndrome.
A 16-year-old Iranian boy presented with a one year history of skin lesions in knees and elbows was diagnosed of psoriasis disease. He had a history of recurrent infections including otitis media, pneumonia, diarrea and skin infection. Laboratory results showed increased level of total IgE and normal in other immunoglobulin. Histologic finding showed hyperkeratosis, parakeratosis of acanthotic epidermis with regular elongation of rete ridges diagnose psoriasis disorder.
In conclusion, this is the first case of hyper IgE patient with psoriasis disorder. We addressed the important laboratory findings and actual theories explaining possible association between hyper IgE immunoglobulinemia and psoriasis disorder.
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，被描述为乔布氏综合征，其特征为血清IgE水平升高、湿疹、反复的皮肤和肺部感染。在本文中，我们报告了一例高免疫球蛋白E综合征病例。
一名16岁的伊朗男孩，有膝关节和肘关节皮肤病变1年病史，被诊断为银屑病。他有反复感染史，包括中耳炎、肺炎、腹泻和皮肤感染。实验室检查结果显示总IgE水平升高，其他免疫球蛋白正常。组织学检查发现有角化过度、棘层肥厚的不全角化， rete嵴规则延长，诊断为银屑病。
总之，这是首例患有银屑病的高免疫球蛋白E患者。我们阐述了重要的实验室检查结果以及解释高免疫球蛋白E血症与银屑病之间可能关联的现有理论。
