Interleukin 1 receptor antagonist deficiency presenting as infantile pustulosis mimicking infantile pustular psoriasis Academic Article uri icon

Overview

MeSH Major

  • Hereditary Autoinflammatory Diseases
  • Interleukin 1 Receptor Antagonist Protein
  • Psoriasis
  • Skin Diseases

abstract

  • Recent identification of DIRA as a disease entity, compounded by the limited number of reported cases, makes early identification difficult. It is critical to consider this entity in the differential diagnosis of infantile pustulosis. Targeted therapy with the recombinant human interleukin 1 receptor antagonist anakinra can be lifesaving if initiated early. A high carrier frequency of the 175-kb DIRA-associated genomic deletion in the Puerto Rican population strongly supports testing infants presenting with unexplained pustulosis in patients from this geographic region.

publication date

  • June 2012

Research

keywords

  • Academic Article

Identity

Language

  • eng

PubMed Central ID

  • PMC3474848

Digital Object Identifier (DOI)

  • 10.1001/archdermatol.2011.3208

PubMed ID

  • 22431714

Additional Document Info

start page

  • 747

end page

  • 52

volume

  • 148

number

  • 6