摘要
先天性糜烂性和水疱性皮病伴网状柔软瘢痕是一种罕见的先天性疾病,目前发病机制尚不明确,常见于早产、宫内感染、单纯疱疹病毒(HSV)感染等。本病的特征性皮损是糜烂和水疱,好发于躯干和四肢,通常累及75以上的体表面积,常在数月内愈合,形成特征性柔软的网状瘢痕。本病可累及其他系统,如神经、心血管及骨骼系统。辅助检查手段包括组织病理、直接免疫荧光和电镜检查等。临床上,本病需要与其他新生儿时期出现糜烂和水疱皮损的皮肤病相鉴别,如先天性HSV感染、先天梅毒、色素失禁症、局灶性真皮发育不良等。本病可在出生后几个月内迅速自愈,无需特殊治疗,对症支持治疗即可。
Congenital erosive and vesicular dermatosis with reticulated supple scarring is a rare congenital disorder with unknown aetiology.It is often observed in premature infants,intrauterine infection cases,and neonates with herpes simplex virus(HSV)infections.It is characterized by vesicles and erosions,which are commonly found on the trunk and extremities,typically involving greater than 75 of the skin surface that heal with distinctive supple and reticulated scarring in a few months.Systemic involvement may affect the nervous,cardiovascular,and skeletal systems.Auxiliary examination methods include pathological biopsy,direct immunofluorescence,and electron microscopy.Differential diagnosis must be distinguished from other neonatal vesicular-erosive dermatoses,such as congenital HSV infection,congenital syphilis,incontinentia pigmenti,and focal dermal hypoplasia.The condition typically resolves spontaneously within the first few months of life,rarely requiring targeted therapeutic interventions.Management primarily focuses on symptomatic relief and supportive treatments.
作者
马锦媛
魏爱华
MA Jinyuan;WEI Aihua(Department of Dermatology,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China)
出处
《中国皮肤性病学杂志》
北大核心
2025年第8期834-837,共4页
The Chinese Journal of Dermatovenereology
基金
国家自然科学基金面上项目(82373505,82173447)。
