Low-level NLRP3 mosaicism extends Schnitzler phenotypic spectrum

Daskalopoulou et al. (2025) detected low-level somatic NLRP3 mosaicism in four patients with chronic urticarial lesions including Schnitzler syndrome cases, extending the phenotypic spectrum of NLRP3-related disorders. Findings support considering NLRP3 inhibitor therapy in select Schnitzler-spectrum patients.