Morren M.,UZ Leuven Leuven |
Flour M.,UZ Leuven Leuven |
Garmyn M.,UZ Leuven Leuven
Nouvelles Dermatologiques | Year: 2012
Hereditary epidermolysis bullosa is a heterogenous group of disorders varying from mild to very severe and life threatening, in which the anchrage of epidermis to dermis is deficient. Blister and bullae formation of skin and mucosae after minor trauma is the most important clinical manifestation. In the dystrophic forms these blisters heal with scarring leading to mitten hand deformities. Moreover, in the most severe forms, systemic manifestations like severe therapy resistant anemia, oesophageal strictures, constipation, cornea ulcers, renal insufficiency, cardiomyopathy... are also present. On skin with recurrent blistering atypical naevocellular naevi appear and spinocellular epithelioma which have a bad prognosis, despite of their well differentiated appearance. Treatment at this moment is symptomatic, with measures to prevent bulla formation, and woundcare with special minimally adhesive, bandages. There is hope that treatments based on reparing the genetic defect might bring better perspectives for these patients in the near future. © Nouv Dermatol 2012.