Consistently, many psoriasis susceptibility genes encode the TRAF6 signaling players, such as ACT1 (<i>TRAF3IP2</i>), A20 (<i>TNFAIP3</i>), ABIN1 (<i>TNIP1</i>), IL-36Ra (<i>IL36RN</i>), IkappaBzeta (<i>NFKBIZ</i>), and CARD14.
Previous studies reported that the interleukin-36 (IL-36) cytokines [IL-36α, IL-36β, IL-36γ and IL-36 receptor antagonists (IL-36RA)] are important players in the pathogenesis of psoriasis (PS).
These studies found that IL36RN mutations account for only a fraction of GPP cases presenting with concomitant psoriasis vulgaris (PV; common or typical psoriasis).
We identified a novel homozygous missense mutation in IL36RN in two siblings, and showed the molecular basis of the condition to be both distinct from psoriasis and distinct between the two families studied.