Via practica 4/2016

Epidermolysis bullosa from the perspective of a plastic surgeon

Epidermolysis bullosa (EB) is ranked among a group of rare heritable blistering disorders. EB is characterized by fragility of the skin and blister formation, following minor trauma or traction on the skin. The generalised recessive dystrophic form of epidermolysis bullosa is the most serious type. Interdigital pseudosyndactyly and hand contracture are common complications of pediatric patiens. Formation of skin cancer – especially squamous cell carcinoma - is characteristic in adults with EB. The purpose of the article is to describe treatment of EB patiens by plastic surgeon based on thirty years of head author’s experience.

Keywords: epidermolysis bullosa, EB, pseudosyndaktylie, flekční kontraktura, spinocelulární karcinom