Epidermolysis bullosa: prospects for cell-based therapies

J Invest Dermatol. 2008 Sep;128(9):2140-2. doi: 10.1038/jid.2008.216.

Abstract

Heritable forms of epidermolysis bullosa (EB) are characterized by chronic, lifelong blistering and erosions due to mutations in 10 distinct genes expressed at the cutaneous basement membrane zone. No specific treatment for this group of intractable diseases is currently available. Recent progress in molecular therapies has indicated that cell-based approaches may potentially offer amelioration--and perhaps even cure--for afflicted individuals. In this issue, Wong et al. (2008) demonstrate the feasibility of direct intradermal injection of allogeneic fibroblasts to the lesional skin of patients with recessive dystrophic EB, with improvement in skin fragility.

Publication types

  • Comment

MeSH terms

  • Biopsy
  • Cell- and Tissue-Based Therapy / methods*
  • Collagen Type VII / genetics
  • Collagen Type VII / metabolism
  • Epidermolysis Bullosa / genetics
  • Epidermolysis Bullosa / metabolism
  • Epidermolysis Bullosa / therapy*
  • Fibroblasts / cytology
  • Fibroblasts / metabolism
  • Fibroblasts / transplantation*
  • Humans
  • Skin / metabolism
  • Skin / pathology
  • Transplantation, Homologous

Substances

  • Collagen Type VII