Two cases of Dupilumab responsive Kimura disease

Abstract

Abstract Kimura disease (KD) is a rare, chronic angio-lymphoproliferative inflammatory disease appearing to be mostly restricted to the skin and soft tissue. Cutaneous involvement of KD includes head and/or neck nodules showing suggestive histological features, frequently associated with atopic dermatitis-like or prurigo-like presentation. KD is challenging to treat, with high rate of recurrence using current therapeutical strategies. Evidence for a type-2 immunity (Th2) involvement in KD pathogenesis has been found in previous studies. Consequently, this study aimed to determine the efficacy and safety of dupilumab, a human monoclonal antibody inhibiting the Th2-key interleukine-4 and -13 signalling, within a single-centre cohort of cutaneous KD.Two adults with a diagnosis of refractory (failure of at least one treatment line) cutaneous-restricted KD based on clinical, biological, histological, molecular and imaging findings received dupilumab for KD and showed dramatic response and a good safety profile.