Two cases of dupilumab-responsive Kimura disease.

Kimura disease (KD) is a rare, chronic angiolymphoproliferative 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 an atopic dermatitis-like or prurigo-like presentation. KD is challenging to treat, with high rate of recurrence using current therapeutic strategies. Evidence for involvement of a T-helper type 2 (Th2) immune response 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 that inhibits signalling of key Th2 cytokines, interleukin (IL)-4 and IL-13, within a single-centre cohort of patients with 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 with a good safety profile.
Competing Interests: Conflicts of interest G.B. has consulted for Novartis (board). M.J. is an investigator and medical/scientific advisor for Sanofi. A.P. has received honoraria from Sanofi as a speaker. M.B. has received grants/research support from Kyowa kirin and Takeda; and has received honororia or consultation fees from Bristol Myers Squibb, Cerba Research, Innate Pharma, Kyowa kirin, L’Oréal, Takeda and Sanofi. J.D.B. is an investigator and medical/scientific advisor for Sanofi. T.M. has consulted for Janssen, Novartis and Sanofi (boards). The remaining authors declare no conflicts of interest.
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