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

Volker-Touw, C. M., de Koning, H. D., Giltay, J., De Kovel, C. G. F., van Kempen, T. S., Oberndorff, K., Boes, M., van Steensel, M. A., van Well, G. T., Blokx, W. A., Schalkwijk, J., Simon, A., Frenkel, J., & van Gijn, M. E. (2017). Erythematous nodes, urticarial rash and arthralgias in a large pedigree with NLRC4-related autoinflammatory disease, expansion of the phenotype. British Journal of Dermatology, 176(1), 244-248. doi:10.1111/bjd.14757.
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.

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Publication type
Journal article
Publication date
2017
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