Dominant dystrophic epidermolysis bullosa with congenital absence of skin and brachydactyly of the great toes

Pediatr Dermatol. 2021 Sep;38(5):1251-1254. doi: 10.1111/pde.14727. Epub 2021 Aug 2.

Abstract

Epidermolysis bullosa (EB) encompasses a phenotypically and genetically heterogeneous group of inherited skin disorders characterized by blistering and erosions of the skin with minimal trauma. Dystrophic EB (DEB), both dominant and recessive, can be associated with several extracutaneous manifestations, including musculoskeletal deformities. Congenital deformities of the feet have rarely been reported in the literature. We describe an infant with dominant DEB and congenital absence of the skin who presented with congenital brachydactyly of the bilateral great toes.

Keywords: brachydactyly; dermatology; epidermolysis bullosa; epidermolysis bullosa dystrophica; musculoskeletal abnormalities.

Publication types

  • Case Reports

MeSH terms

  • Brachydactyly*
  • Epidermolysis Bullosa Dystrophica* / complications
  • Epidermolysis Bullosa Dystrophica* / diagnosis
  • Epidermolysis Bullosa Dystrophica* / genetics
  • Epidermolysis Bullosa*
  • Hallux*
  • Humans
  • Infant
  • Skin