Juvenile interleukin-36 receptor antagonist deficiency (DITRA) with c.80T>C (p.Leu27Pro) mutation successfully treated with etanercept and acitretin

General pustular psoriasis (GPP) is a rare form of psoriasis and is clinically characterized by widespread eruptions of sterile pustules and bright erythematous skin accompanied by periods of fever, chills, rigors, neutrophilia, and elevated serum C-reactive protein.1 Acrodermatitis of Hallopeau, palmoplantar psoriasis pustulosis, and annular pustular psoriasis may be variations of this GPP.2 In 2011, Marrakchi et al3 reported a subgroup of GPP patients with a specific genetic defect: a deficiency of interleukin-36 receptor antagonist (DITRA).