Linkage of bacterial colonization of skin and the urticaria-like rash of NLRP3-mediated autoinflammatory syndromes through mast cell-derived TNF-α

Y Nakamura, N Kambe - Journal of Dermatological Science, 2013 - Elsevier
As anti-cytokine therapies were developed for clinical use during the last decade, anti-IL-1
therapies have emerged as a highly effective treatment for cryopyrin-associated periodic
syndromes (CAPSs), including the “urticaria-like rash.” Based on clinical observations, we
hypothesized that NLPR3 activation and IL-1β production, in particular in mast cells (MCs),
are important for the development of this eruption, due to the observation that CAPS patients
have gain-of-function mutations in NLRP3 that result in unregulated excess levels of IL-1β …