”Trap” the diagnosis: a man with recurrent episodes of febrile peritonitis

Isr Med Assoc J. 2012 Apr;14(4):229-31.
Arad U, Niv E, Caspi D, Elkayam O.

Monogenic periodic fever syndromes are characterized by recurrent episodes of
fever, accompanied by localized inflammatory manifestations. Among them, familial
Mediterranean fever (FMF) is the most studied and is by far the most prevalent
periodic fever syndrome in Israel. We present a diagnostic workup of a patient
suffering from a periodic fever syndrome, initially thought to be FMF and
characterized by attacks of fever, severe abdominal pain, a migratory
erythematous rash and conjunctivitis. The development of periorbital edema
presenting as diplopia led to consideration of tumor necrosis factor
receptor-1-associated periodic syndrome (TRAPS). Genetic tests confirmed the
diagnosis. This case should alert us that even in Israel, a patient with periodic
fever not fully consistent with the typical features of FMF, should be evaluated
for other periodic fever syndromes.