Epidermolysis bullosa in South Africa

The term 'epidermolysis bullosa hereditaria' is applied to a heterogeneous group of mechanobullous diseases of which at least 19 different forms have been reported. Classification of these separate genetic entities into various subtypes is possible on clinical, genetic, biochemical and histopathological bases. Some forms are potentially lethal, while others are benign. Many of the subgroups are represented in South Africa, although little has been published locally on these conditions. On the basis of a wide-scale study in the RSA, the condition is reviewed in order to present current concepts and future developments of this rare but significant genodermatosis.