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Figure 1: Left - A) maculopapular rash with target lesions in upper limbs at initial clinical presentation; B) maculopapular rash with target lesions in both upper and lower limbs during relapse. Right – C) Overview of the NLRP12 activity in the canonical and non-canonical NF-KB pathways, showing NLRP12 role in inhibiting IRKA1 and IKK, leading to the control in the production of inflammatory cytokines, like IL-1β. Loss of function (LoF) mutations stop that inhibition leading to an increased transcription of pro-inflammatory cytokines, which promotes inflammation; D) Role of IL-1 receptor antagonist (IL-1RA) as therapeutic drug that blocks the IL-1 receptor (IL-1R), inhibiting the action of IL-1 and controlling inflammation in these patients.
For this manuscript Figure 1 Right was created with BioRender.com and a publication license was obtained.
Table 1: Laboratory studies: immunological and infectious diseases screening during hospital admission