Stevens-Johnson-Syndrom/toxisch epidermale Nekrolyse – Diagnostik und Therapie aus ophthalmologischer Sicht

Zusammenfassung

Beim Stevens-Johnson-Syndrom (SJS) bzw. der toxisch epidermalen Nekrolyse (TEN) handelt es sich um eine sehr seltene, schwere und häufig lebensbedrohliche Erkrankung von Haut und Schleimhäuten. Je nach Schweregrad spricht man von einem Stevens-Johnson-Syndrom (< 10% betroffene Körperoberfläche), Übergangsformen (10 – 30% betroffene Körperoberfläche) und von der toxisch epidermalen Nekrolyse (> 30% betroffene Körperoberfläche). In der akuten Phase der Erkrankung können bei 80% der Betroffenen die Augen mitbeteiligt sein. Dabei ist ein konsequentes interdisziplinäres diagnostisches und therapeutisches Vorgehen in der Akutphase von besonderer Bedeutung, um das Ausmaß der Schädigung der Augenoberfläche möglichst gering zu halten. Hierbei kommt neben der topischen Behandlung dem Einsatz von humaner Amnionmembran eine besondere Bedeutung zu. Langzeitschäden durch chronische Vernarbungen und ausgeprägte Trockenheit der Augen treten trotz aller Maßnahmen bei bis zu 50% der Betroffenen auf und sind nicht selten die schwerwiegendste Komplikation im Langzeitverlauf. Um auf lange Sicht eine Erblindung zu vermeiden, kommen neben einer topischen Basistherapie in erster Linie Korrekturoperationen von Lidfehlstellungen und Sklerallinsen zum Schutz der Augenoberfläche infrage. Die Indikation zur Hornhauttransplantation im Sinne einer allogenen perforierenden Limbokeratoplastik und Keratoprothesen sollte aufgrund der sehr schlechten Prognose zurückhaltend gestellt werden.

Schlüsselwörter

Bindehaut - Kornea - Lider

Publication History

Received: 20 January 2025

Accepted: 13 February 2025

Article published online:
16 June 2025

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