Autoinflammatory diseases are characterized by abnormal systemic inflammatory responses primarily mediated by a dysfunction of the innate immunity. Disease prototypes are the rare cryopyrin-associated periodic syndromes (CAPS), which are caused by mutations of the...
Schnitzler’s syndrome is a rare autoinflammatory disorder characterized by interleukin-1ß-mediated and neutrophil-dominated inflammation. Neutrophil extracellular traps (NETs) are web-like structures of decondensed chromatin, histones, and antimicrobial peptides...
Coagulation factor XII (FXII) drives production of the inflammatory peptide bradykinin. Pathological mutations in the F12 gene, which encodes FXII, provoke acute tissue swelling in hereditary angioedema (HAE). Interestingly, a recently identified F12 mutation, causing...
Urticarial skin reactions are one of the most frequent problems seen by allergists and clinical immunologists in daily practice. The most common reason for recur- rent wheals is spontaneous urticaria. There are, however, several less common diseases that present with...
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