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Two dominant periodic fevers, tumour necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndrome (CAPS), are prominent auto-inflammatory disorders. Mutations in the 55 kDa TNF receptor superfamily 1A gene (TNFRSF1A) and in the cold-induced auto-inflammatory syndrome 1 gene (CIAS1) were recently associated with TRAPS1 and CAPS,2 respectively. Following publications describing the efficacy of etanercept (enbrel), a recombinant human TNF receptor 1B fusion protein in patients with TRAPS3 and anakinra (kineret), a recombinant human interleukin-1 (IL1)-receptor antagonist, in patients with CAPS,4 targeted biotherapies have been repeatedly and successfully given for these two conditions.
Our proband (fig 1) is a 36-year-old French woman who presented with rashes of urticaria and oedema precipitated by exposure to heat and water. These rashes, lasting a …
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