Abstract
Objective To explore the etiology, classification, treatment and prognosis of congenital absence of skin so as to improve its management. Methods Retrospective analyses were performed for the clinical characteristics and treatment in 3 cases of congenital absence of skin at our hospital. And 305 cases of congenital absence of skin were collected from the literature. Results Three cases of skin defect healed by parchment-like scar tissue. And there was neither contracture nor dysfunction. Among 305 cases, there were 8 deaths and 234 cases received conservative treatments. And 5/63 surgical cases were affected by scar contracture. The mortality and disability rates were low. Conclusions The etiology of congenital skin defect has remained elusive. Superficial wound may be managed conservatively. Cases of bone exposure, vital organ exposure and deep defect should be operated. If treated properly, the prognosis is excellent without any significant impact of daily life. Key words: Absence of skin; Epidermolysis bullosa; Cicatrix
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