Epidermolysis bullosa pruriginosa

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Abstract

Epidermolysis bullosa (EB) pruriginosa is a rare clinical subset of dystrophic EB, characterized by marked itching and presence of prurigo-like or lichenoid features. In order to further delineate the phenotype and understand the pathogenesis of this disorder, the clinical, histological and ultrastructural findings of a 19-year-old patient presenting a typical form of EB pruriginosa are described. The prevalence of papular itchy lichenoid lesions, signs of scratching and paucity of blisters at the time of clinical examination may result in incorrect diagnosis and treatment. Microscopic studies of the lesions show the typical 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 abnormal dermal reactivity of some patients to their inherited bullous disorder.

Original languageEnglish
Pages (from-to)65-68
Number of pages4
JournalDermatology
Volume195
Issue number1
Publication statusPublished - 1997

Keywords

  • Collagen
  • Dystrophic epidermolysis bullosa
  • Electron microscopy
  • Genetic skin disease
  • Prurigo

ASJC Scopus subject areas

  • Dermatology

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