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Deficiency of the interleukin-36 receptor antagonist (DITRA) is an autosomal recessive disease caused by mutations of IL36RN gene.1 Patients suffer from flares of acute generalised pustular psoriasis and systemic inflammation. We present two paediatric cases of DITRA with a severe clinical course, resistant to multiple therapies in whom the use of high doses of ustekinumab (a monoclonal antibody against the p40 subunit of both IL-12 and IL-23) lead to a persistent control of the disease.
A 4-year-old boy, born from unrelated parents, presented at the age of 3 years with inverse psoriasis in the genital area. After some months, he developed diffuse pustular lesions associated with fever, elevation of acute phase reactants and poor general condition, requiring parenteral antibiotics and high-dose steroids (figure 1). Compound heterozygosity for the IL36RN P76L/S113L mutations was detected. Different treatments (acitretin, high-dose ciclosporin, …
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