EPIDERMOLYSIS-BULLOSA PRURIGINOSA

Epidermolysis bullosa (EB) pruriginosa is a rare clinical subset of dy strophic EB, characterized by marked itching and presence of prurigo-l ike or lichenoid features. In order to further delineate the phenotype and understand the pathogenesis of this disorder, the clinical, histo logical and ultrastructural findings of a 19-year-old patient presenti ng a typical form of EB pruriginosa are described. The prevalence of p apular itchy lichenoid lesions, signs of scratching and paucity of bli sters at the time of clinical examination may result in incorrect diag nosis and treatment. Microscopic studies of the lesions show the typic al findings of dystrophic EB associated with an unusually high density of collagen bundles and absence of elastic fibres in the upper dermis . Itching lichenoid lesions of EB pruriginosa could represent an abnor mal dermal reactivity of some patients to their inherited bullous diso rder.