ABSTRACT
Although probably existing since ancient times, familial Mediterranean
fever (FMF) was not recognized as a unique entity until 1945, when Siegal
(1) described 10 cases of ‘‘benign paroxysmal peritonitis.’’ Since then, this
condition has been given a variety of names (e.g., hereditary recurrent polyserositis, periodic disease, Armenian disease), emphasizing its genetic and
episodic nature and the geographic distribution. FMF is characterized by
recurrent episodes of fever and serositis, involving primarily the peritoneum
(affecting 95% of patients), the pleura (40% of patients) and the synovial membrane of the joints (75% of patients). Typical febrile episodes of skin rash (erysipelas-like erythema), pericarditis, scrotal pain, and myalgia occur
less frequently. The poorly understood leg pain, together with the rare
manifestation of protracted febrile myalgia, the uncommon development of chronic arthritis and the nephropathic AA amyloidosis complete the
clinical spectrum (2).
