Epidermolysis Bullosa Pruriginosa: Case Series and Review of the Literature

Int J Low Extrem Wounds. 2015 Jun;14(2):196-9. doi: 10.1177/1534734615572469. Epub 2015 Feb 17.

Abstract

Epidermolysis bullosa (EB) pruriginosa is a rare clinical subtype of dystrophic epidermolysis bullosa (DEB) that is characterized by intense pruritus resulting in hypertrophic, lichenified, prurigo-like plaques and nodules secondary to scratching. The variability in the age of onset, rarity of intact bullae, histologic ambiguities, and close resemblance to other conditions such as acquired inflammatory dermatoses may make diagnosis difficult for this unusual condition, for which fewer than 100 cases have been documented. In this report, we describe 3 cases of EB pruriginosa and review the current literature.

Keywords: case series; dominant dystrophic epidermolysis bullosa; epidermolysis bullosa pruriginosa; literature review.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Biopsy
  • Child
  • Diagnosis, Differential
  • Epidermolysis Bullosa Dystrophica / diagnosis*
  • Female
  • Humans
  • Leg
  • Male
  • Middle Aged
  • Skin / pathology*

Supplementary concepts

  • Epidermolysis Bullosa Pruriginosa