Thelansis

  Recessive dystrophic epidermolysis bullosa (RDEB) is a subgroup of hereditary blistering diseases characterized by repetitive wounding and healing with subsequent extensive scarring. Is a group of genetic disorders of the skin characterized by marked fragility and a tendency to form blisters and erosions in response to minor trauma. There are more than 12 hereditary types of EB, which are subclassified into four major forms: dominant EB simplex, recessive junctional EB, dominant dystrophic EB, and recessive dystrophic EB. Dystrophic forms of EB feature repetitive wounding and healing of the skin associated with severe scarring. A special feature of RDEB patients is a tendency to develop aggressive squamous cell carcinomas in areas of severe scarring. These cancers lead to high morbidity and mortality at a relatively early age. The pathogenesis of RDEB remains unclear; however, two hypotheses have been extensively studied. One hypothesis is that the RDEB skin has a rudimentary o...