Standard dose of Ustekinumab for childhood-onset deficiency of interleukin-36 receptor antagonist

We read with interest the letter from Bonekamp et al 1 reporting two children with severe deficiency of interleukin-36 receptor antagonist (DITRA) treated with high dose of Ustekinumab. DITRA, an autosomal recessive disease caused by mutations of IL36RN , is characterised by generalised pustular psoriasis and systemic inflammation.2 We report here a paediatric case successfully treated with standard dose of Ustekinumab, a monoclonal antibody against the p40 subunit of interleukin (IL)-12/IL-23. We reported in 2015 a boy born from consanguineous Tunisian parents who presented at 1 month of life with diffuse inflammatory pustular psoriasis and erythrodermic extension.3 At the age of 2 months, he was referred to us with fever, irritability, severe failure to thrive and …