Prurigo-like atopic dermatitis in a child with CARD11-associated severe combined immunodeficiency successfully treated with dupilumab

Pediatr Dermatol. 2024 Jan-Feb;41(1):158-159. doi: 10.1111/pde.15453. Epub 2023 Oct 27.

Abstract

A 12-year-old boy affected by severe combined immunodeficiency due to a heterozygous variant in the CARD domain of CARD11, c.169G>A; p.Glu57Lys, developed severe atopic dermatitis and alopecia areata. After failure of conventional systemic therapy, dupilumab was administered at a dose of 400 mg subcutaneously, followed by 200 mg every 14 days. The patient had an excellent clinical response after 1 month and complete remission after a year, with the absence of side effects, demonstrating good efficacy and safety profile.

Keywords: CARD11; atopic dermatitis; dupilumab; immunodeficiency.

Publication types

  • Case Reports
  • Letter

MeSH terms

  • Antibodies, Monoclonal, Humanized / therapeutic use
  • CARD Signaling Adaptor Proteins / genetics
  • Child
  • Dermatitis, Atopic* / complications
  • Dermatitis, Atopic* / drug therapy
  • Guanylate Cyclase
  • Humans
  • Male
  • Prurigo* / drug therapy
  • Severe Combined Immunodeficiency* / complications
  • Severity of Illness Index
  • Treatment Outcome

Substances

  • dupilumab
  • Antibodies, Monoclonal, Humanized
  • CARD11 protein, human
  • Guanylate Cyclase
  • CARD Signaling Adaptor Proteins