Efficacy of Dupilumab for controlling severe atopic dermatitis with dominant-negative CARD11 variant.

Hypomorphic dominant-negative mutations in Caspase Recruitment Domain-containing Protein 11 (CARD11) is a rare cause of severe atopic dermatitis. CARD11 is a membrane protein involved in TCR signalling via the NF-kB pathway that has been identified as a predisposition locus for severe atopy. A loss of function activity of CARD11 due to a dominant-negative effect of CARD11 variant leads to severe atopic dermatitis associated with a defective T cell response that polarize toward a Th2 phenotype. We report the successful treatment of severe atopic dermatitis with dominant-negative CARD11 variant with Dupilumab.