罕见先天性皮肤发育不全的保守治疗1例。

Luis Chang-Azancot, Manuel Campello, Javier Montón
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引用次数: 1

摘要

摘要先天性皮肤发育不全(ACC)是一种罕见的先天性皮肤、真皮附属物和皮下组织的局部缺失。ACC的病因尚不清楚,但遗传是最被广泛接受的原因。我们报告一例罕见的足月(37 + 5周)女性新生儿,其上肢和下肢局部皮肤完全缺失。患者被诊断为ACC伴大疱性表皮松解症(EB;(一种容易引起皮肤起泡的疾病),最初使用保守措施治疗。我们每天使用莫匹罗星外用软膏和浸渍水胶体和凡士林的非闭塞聚酯网。3周后患处完全愈合。管理ACC患者通常具有挑战性,根据病变的严重程度,该方法可能包括手术和保守治疗。我们的病例报告表明,保守的方法可以有效地管理某些类型的ACC和EB病变。然而,需要进一步的研究来更好地了解这种实体的发病机制和最佳管理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Conservative Management of a Rare Entity-Aplasia Cutis Congenita: A Case Report.

Aplasia cutis congenita (ACC) is a rare entity characterized by a congenital localized absence of the skin, dermal appendages, and subcutaneous tissue. The cause of ACC is not clear, but inheritance is the most widely accepted cause. We report a rare case of a full-term (37 + 5 weeks) female newborn who showed complete absence of skin in localized areas of the upper and lower extremities. The patient was diagnosed with ACC associated with epidermolysis bullosa (EB; a disease that causes the skin to blister easily) and was initially treated using conservative measures. We applied mupirocin topical ointment and a nonocclusive polyester mesh impregnated with hydrocolloid and petroleum jelly daily. Complete healing of the affected areas occurred after 3 weeks. Managing patients with ACC is often challenging and based on the severity of the lesions, the approach may include both surgical and conservative treatment. Our case report suggests that a conservative approach can be effective for managing certain types of ACC and EB lesions. However, further research is warranted to better understand the pathogenesis and optimal management of this entity.

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