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Erythematous nodes, urticarial rash and arthralgias in a large pedigree with NLRC4-related autoinflammatory disease, expansion of the phenotype

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Citation

Volker-Touw, C. M., de Koning, H. D., Giltay, J., De Kovel, C. G. F., van Kempen, T. S., Oberndorff, K., et al. (2016). Erythematous nodes, urticarial rash and arthralgias in a large pedigree with NLRC4-related autoinflammatory disease, expansion of the phenotype. British Journal of Dermatology. Advance online publication. doi:10.1111/bjd.14757.


Cite as: http://hdl.handle.net/11858/00-001M-0000-002B-9C42-2
Abstract
Autoinflammatory disorders (AID) are a heterogeneous group of diseases, characterized by an unprovoked innate immune response, resulting in recurrent or ongoing systemic inflammation and fever1-3. Inflammasomes are protein complexes with an essential role in pyroptosis and the caspase-1-mediated activation of the proinflammatory cytokines IL-1β, IL-17 and IL-18.