肢端先天性皮肤增生症(第 VII 组):尼日利亚西北部的病例报告。

Sanni Abiola Usman, Lawal Olatunde Taslim, Muhammad Ibrahim Habib, Musa Tawakaltu Lily, Olateju Eyinade Kudirat, Audu Isah Lamidi
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摘要

先天性皮肤缺失症(ACC)是一种罕见的先天性疾病,其特征是局部或大面积皮肤缺失,主要影响头皮。双侧上下肢均受累的情况并不常见。这是一例罕见先天性疾病的病例报告。患者是一名 26 小时大的男婴,因上下肢皮肤广泛缺失而入院。他与整形外科团队共同接受了保守治疗,治疗范围为 ACC 第 VII 组。在入院 12 天前,他的皮损愈合情况令人满意,但在父母的劝说下,他还是违背医嘱签了字。上下肢均受累的 ACC 是一种罕见的表现,对保守治疗反应良好。报告强调,需要制定一个法律框架,以便医生在不符合儿童最佳利益的情况下推翻看护人的决定。
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Aplasia cutis congenita of extremities (group VII): case report from North-Western Nigeria.

Aplasia cutis congenita (ACC) is a rare congenital disorder characterised by localised or widespread absence of skin mainly affecting the scalp. Bilateral involvement of both the upper and lower extremities is uncommon. This is a case report of a rare congenital disorder. The patient was a 26-hour-old male baby admitted with extensive absence of skin on the lower and upper extremities. He was co-managed conservatively with the plastic surgical team for ACC group VII. The lesions were healing satisfactorily until 12 days into the admission when the parents signed against medical advice despite counselling. ACC with involvement of both upper and lower extremities is a rare presentation that responds to conservative treatment. The report emphasises the need for a legal framework for physicians to override the decision of the caregiver not in the best interest of a child.

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