Psoriasis Histology — SCE Dermatology MCQ

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HardInflammatory DermatosesPsoriasis HistologySCE Dermatology

A child has recurrent episodes of sterile generalised pustulation, fever and systemic inflammation from early infancy. Which gene is most strongly associated with deficiency of the interleukin-36 receptor antagonist?

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Correct answer: D — IL36RN

Biallelic IL36RN variants cause deficiency of the interleukin-36 receptor antagonist and can produce severe early-onset generalised pustular psoriasis. Other variants can contribute to pustular phenotypes, but IL36RN directly encodes the deficient antagonist.

Reference: https://www.nice.org.uk/guidance/cg153/chapter/recommendations