Die operativen Behandlungsmöglichkeiten der Epidermolysis bullosa hereditaria

Abstract

After demonstrating four clinical patterns of epidermolysis bullosa hereditaria the operative procedure will be discussed in two cases. One is a now 16-year-old male, the other a 18-year-old girl, who suffer from this disease.
While removing the affected skin in a first operation, the defect is covered by a split skin graft taken from a non-affected area.
In a second operation or in repeated operative procedures, not only must the affected skin be removed but also any existing scars and contractures.
Children who suffer from this form of epidermolysis bullosa hereditaria present a severe problem.
For all who deal with this problem, the painstaking preoperative preparation as well as the choice of the most suitable anaesthetic are as important in every respect as the operation itself and the postoperative and psychosocial care.

Zusammenfassung

Kinder mit der schweren Form der Epidermolysis bullosa hereditaria sind für alle Beteiligten ein schwieriges Problem. Sorgfältige präoperative Vorbereitung, sowie die Wahl des geeigneten Narkosemittels sind ebenso wichtig, wie die Operation selbst und die postoperative Behandlung und Betreuung. Frühzeitige aktive Übungsbehandlung unter sachkundiger Anleitung und die aktive Mithilfe der Eltern erscheinen ein ansprechendes Ergebnis zu gewährleisten.

Key words

Epidermolysis bullosa hereditaria, dermatogen

Schlüsselwörter

Epidermolysis bullosa hereditaria, Hautveränderungen