Epidermolysis bullosa pruriginosa treated with dupilumab

Pediatr Dermatol. 2021 Mar;38(2):526-527. doi: 10.1111/pde.14493. Epub 2020 Dec 18.

Abstract

Epidermolysis bullosa pruriginosa (EBP) is a variant of dystrophic epidermolysis bullosa characterized by intense pruritus and prurigo nodularis-like lesions. While medical therapies for EBP exist, current treatments are not consistently effective, and symptoms often cause decreased quality of life. Here, we report two cases of EBP treated with dupilumab, which decreased symptoms of pruritus and improved skin findings. Both patients have been on dupilumab for over one year with sustained improvement and no adverse effects; although in one patient, increased dosing was required to attain optimal control of disease.

Keywords: dupilumab; epidermolysis bullosa dystrophica; epidermolysis bullosa pruriginosa; pruritus; therapy-systemic.

MeSH terms

  • Antibodies, Monoclonal, Humanized
  • Epidermolysis Bullosa Dystrophica* / drug therapy
  • Humans
  • Pruritus
  • Quality of Life

Substances

  • Antibodies, Monoclonal, Humanized
  • dupilumab

Supplementary concepts

  • Epidermolysis Bullosa Pruriginosa