Response to Interleukin (IL)-17 Inhibition in an Adolescent With Severe Manifestations of IL-36 Receptor Antagonist Deficiency (DITRA)

Deficiency of the interleukin (IL)-36 receptor antagonist (DITRA) is a recently described autosomal recessive auto-inflammatory syndrome caused by mutations in the IL36RN gene, characterized clinically by recurrent episodes of generalized skin pustulation, fever, systemic inflammation and leukocytosis.^1,2 A uniformly effective treatment for DITRA has not been identified.