Yaşar Şirin, Yaşar Bulent, Cebeci Filiz, Bayoğlu Duygu, Nuhoğlu Çağatay
Associate Professor; Haydarpasa Numune Training and Research Hospital, Department of Dermatology, Istanbul, Turkey.
Associate Professor; Başkent University, Department of Gastroenterology, Istanbul, Turkey.
J Wound Care. 2018 Nov 2;27(11):768-771. doi: 10.12968/jowc.2018.27.11.768.
Bart syndrome consists of aplasia cutis congenita (ACC) and dominant or recessive dystrophic epidermolysis bullosa (DEB), associated with skin fragility and nail dysplasia. ACC in DEB is thought to be caused by trauma, the most cited cause being in utero formation of bullae consequent to friction of the limbs. Epidermolysis bullosa (EB) refers to a hereditary mechanobullous disease following trauma, characterised by formation of blisters on the skin and mucous membranes. There are four categories of the disease, including epidermolysis bullosa simplex, junctional epidermolysis bullosa, dystrophic epidermolysis bullosa and Kindler syndrome. Infection, sepsis and death may occur as a consequence of generalised blistering with complication. We present the case of a newborn diagnosed with DEB and whose lesions became almost fully epithelialised after treatment with 10% topical sucralfate.
巴特综合征由先天性皮肤发育不全（ACC）和显性或隐性营养不良性大疱性表皮松解症（DEB）组成，伴有皮肤脆弱和指甲发育异常。DEB中的ACC被认为是由创伤引起的，最常被提及的原因是子宫内肢体摩擦导致大疱形成。大疱性表皮松解症（EB）是指一种创伤后遗传性机械性大疱病，其特征是皮肤和黏膜上形成水疱。该疾病有四类，包括单纯性大疱性表皮松解症、交界性大疱性表皮松解症、营养不良性大疱性表皮松解症和金德勒综合征。广泛性水疱形成并伴有并发症可能导致感染、败血症和死亡。我们报告了一例被诊断为DEB的新生儿病例，其皮损经外用10%硫糖铝治疗后几乎完全上皮化。
