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Many autoinflammatory disorders have been identified as genodermatoses that result in increases in certain inflammatory mediators in the patient's circulation. Identification of these abnormalities can, in some instances, result in control of the disorder with an appropriately selected therapy (e.g., the use of anakinra for NOMID). STING (standing for Stimulator of Interferon Genes) has been identified as the cause of a new autoinflammatory syndrome.
These authors noted the new syndrome involving the skin and the lungs accompanied by fever. The cutaneous manifestations in these patients include acral violaceous plaques; nodules on the face, nose, and ears; and ulcerations or gangrene at these sites. They identified six patients with the synd…