[Epidermolysis bullosa. An update].

[kindler syndrome]

Epidermolysis bullosa (EB ) represents a group of diseases characterized by skin fragility usually developing blisters after minimal trauma . The clinical picture ranges from mild subtypes with minor skin reactions to severe forms with lethal outcome within the first months of life . In the severe generalized subtypes , complications such as aggressive squamous cell carcinoma of the skin , anemia , esophageal stenosis and cardiomyopathy can occur so that multidisciplinary patient care is necessary . EB can be divided in four types--EB simplex (EBS ), junctional EB (JEB ), dystrophic EB (DEB ) and Kindler syndrome . All together 33 subtypes can be distinguished . In 2008 a revised EB classification was introduced . Several eponyms for EB subtypes were replaced by descriptive names . The review presents the EB subtypes based on the new EB classification system , the molecular background and new therapeutic options .