Schnitzler’s syndrome; a case highlighting the complications of long-standing acquired autoinflammation

The autoinflammatory syndromes are a family of disorders of dysregulated innate immunity that result in the overproduction of pro-inflammatory cytokines, in particular IL-1β [1]. The protean clinical manifestations of these diseases include: recurrent episodic fever, myalgia, arthralgia and arthritis, general malaise, and atypical urticaria or cutaneous features resembling a number of neutrophilic dermatoses, mainly pyoderma gangrenosum [2, 3]. Autoantibodies are characteristically absent. Symptom [...]