Oral observations of hyper immunoglobulin-e syndrome: A case report

IF 0.6 Q4 DENTISTRY, ORAL SURGERY & MEDICINE
Ibtissam Dahabi, Fatima Zahra Benkarroum, Hakima Chhoul
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引用次数: 0

Abstract

The hyperimmunoglobulin E syndrome is a rare primary immunodeficiency, characterized by a triad of clinical signs including elevated serum IgE levels, recurrent staphylococcal skin abscesses and pneumonia. It can be autosomal dominant or recessive and is characterized by immunological and non-immunological abnormalities.

Individuals with HIES share characteristic facial features, with many oral manifestations like retained deciduous teeth, missing permanent tooth buds, high arched palate, oral and gingival lesions, etc.

This report aimed to describe a rare clinical case of a 13-year-old girl, that was referred to our paediatric dentistry department at the Mohammed V University Hospital in Rabat-Morocco, diagnosed with autosomal dominant hyperimmunoglobulin E syndrome with numerous oral manifestations.

高免疫球蛋白-e综合征的口服观察1例
高免疫球蛋白E综合征是一种罕见的原发性免疫缺陷,其特点是临床症状包括血清IgE水平升高,复发性葡萄球菌性皮肤脓肿和肺炎。它可以是常染色体显性或隐性的,以免疫和非免疫异常为特征。患有HIES的个体具有特殊的面部特征,有许多口腔表现,如乳牙保留,恒牙芽缺失,高弓腭,口腔和牙龈病变等。本报告旨在描述一个罕见的临床病例,一名13岁的女孩,被转介到我们位于摩洛哥拉巴的穆罕默德五世大学医院的儿科牙科部门,诊断为常染色体显性高免疫球蛋白E综合征,伴有许多口腔表现。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Pediatric Dental Journal
Pediatric Dental Journal DENTISTRY, ORAL SURGERY & MEDICINE-
CiteScore
1.40
自引率
0.00%
发文量
24
审稿时长
26 days
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