A case of aplasia cutis type VI (Bart’s syndrome) at University of Abuja Teaching Hospital Gwagwalada, Abuja, Nigeria

Amaka P Ehighibe, A. Abubakar, P. Mshelbwala
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Abstract

Aplasia cutis type VI, commonly referred to as Bart’s syndrome, is a rare genetic disorder characterized by congenital localized absence of skin commonly in the lower limbs, epidermolysis bullosa, and sometimes dystrophic nails. We present a 2-hour-old male infant who presented with congenital absence of skin over both lower limbs at birth. He developed blisters in keeping with epidermolysis bullosa 24 hours after birth. Examination and investigations revealed no systemic involvement. An assessment of aplasia cutis type VI (Bart’s syndrome) was made. He was managed conservatively and wounds showed progressive re-epithelialization.
尼日利亚阿布贾瓜瓦拉达阿布贾大学教学医院6型皮肤发育不全(巴特综合征)1例
VI型皮肤发育不全,通常被称为巴特综合征,是一种罕见的遗传性疾病,其特征是先天性局部皮肤缺失,通常在下肢,大疱性表皮松解症,有时指甲营养不良。我们提出了一个2小时大的男婴谁提出先天性皮肤缺失在出生时,两下肢。他在出生后24小时出现了与大疱性表皮松解症一致的水泡。检查和调查未发现系统性病变。对皮肤发育不全VI型(Bart’s综合征)进行评估。他被保守处理,伤口显示进行性上皮化。
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