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Standard dose of Ustekinumab for childhood-onset deficiency of interleukin–36 receptor antagonist
  1. Bilade Cherqaoui Jr1,2,
  2. Linda Rossi-Semerano1,2,
  3. Maryam Piram1,2,
  4. Isabelle Koné-Paut1,2
  1. 1Paediatric Rheumatology, APHP, CHU Bicêtre, University of Paris Sud, Le Kremlin-Bicetre, Ile-de-France, France
  2. 2CEREMAIA, French Reference Centre for Auto-Inflammatory Diseases and Inflammatory Amyloidosis, Le Kremlin-Bicêtre, Ile-de-France, France
  1. Correspondence to Dr Bilade Cherqaoui Jr, Paediatric Rheumatology, Hopital Bicetre, Le Kremlin-Bicetre, Ile-de-France 94275, France; bilade.cherqaoui{at}hotmail.fr

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We read with interest the letter from Bonekamp et al1 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 …

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