Supplementary Material for: Recurrent Urticaria : A Rare Cryopyrin Associated Periodic Syndrome- Muckle Wells Syndrome

Muckle Wells syndrome is a rare Cryopyrin-Associated Periodic Syndrome (CAPS), an auto-inflammatory disorder due to a loss of function mutation in the NACHT domain of the NLRP3 gene. The loss of cryopyrin activity brought on by this deficiency eventually causes dysregulated inflammation and increased release of the proinflammatory cytokine interleukin (IL)-1 beta. It has an autosomal dominant inheritance. The other two variants of CAPS are neonatal-onset multi-system inflammatory disorder (NOMID) and familial cold auto-inflammatory syndrome (FCAS). MWS mainly presents with recurrent fever, recurrent urticarial rash, sensorineural hearing loss, and secondary amyloidosis, which doesn't respond to the anti-histaminic drugs and steroids. However, anti-IL-1 medications like anakinra, rilonacept, and canakinumab have shown to be highly effective in managing MWS. The etiology and phenotypic presentation of MWS are covered in this case study, followed by clinical perspectives on the illness. With the availability of appropriate medications, future complications can be prevented, and the prognosis is improved.