Başterzi Yavuz, Bağdatoğlu Celal, Sari Alper, Demirkan Ferit
Mersin University Faculty of Medicine, Department of Plastic, Reconstructive and Aesthetic Surgery, Mersin, Turkey.
J Craniofac Surg. 2007 Mar;18(2):427-9. doi: 10.1097/01.scs.0000246500.84935.4f.
Aplasia cutis congenita is a rare congenital disorder of skin and most commonly involves the scalp. The skull and dura underlying the defective skin may also be affected, relative to severity of the disease. The typical lesion is present at birth and ranges in size from 0.5-3 cm. The main complications of larger defects include infection, bleeding and trombosis that may be deadly. Therefore, prompt diagnosis and appropriate treatment are critical for avoiding the adverse outcomes. Controversy exists in the literature regarding the treatment of aplasia cutis congenita; both surgical and conservative treatment modalities have their proponents and opponents. We present a case of full thickness aplasia cutis congenital lesion bigger than 3 cm in diameter that healed with the application of novel wound dressing materials without any complications. The physiopathology, classification and treatment options of the disease are discussed.
先天性皮肤发育不全是一种罕见的先天性皮肤疾病，最常累及头皮。相对于疾病的严重程度，有缺陷皮肤下方的颅骨和硬脑膜也可能受到影响。典型病变在出生时即存在，大小范围为0.5 - 3厘米。较大缺损的主要并发症包括感染、出血和血栓形成，这些可能是致命的。因此，及时诊断和适当治疗对于避免不良后果至关重要。关于先天性皮肤发育不全的治疗，文献中存在争议；手术和保守治疗方式都有其支持者和反对者。我们报告一例直径大于3厘米的先天性全层皮肤发育不全病变病例，应用新型伤口敷料材料后愈合，无任何并发症。本文讨论了该疾病的生理病理学、分类和治疗选择。
