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January 2017

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

Author Affiliations
  • 1Department of Dermatology, University of California–San Francisco School of Medicine, San Francisco
JAMA Dermatol. 2017;153(1):106-108. doi:10.1001/jamadermatol.2016.3490

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.