[Protracted Febrile Myalgia Syndrome with Henoch-Schönlein Purpura: an atypical presentation of Familial...

Familial Mediterranean Fever (FMF) is an hereditary autosomal recessive disease characterized by recurrent attacks of fever, arthritis and serositis: peritonitis, pleurisy and/or pericarditis. Its main complication is systemic AA amyloidosis. The authors present a case of a 8-years-old female child with african ancestry, who was admitted three times since 5 years-old with abdominal pain, fever and high acute phase reactants. At the first admission appendectomy was made and at the third hospital admission the clinical picture was accompanied by myalgia, purpuric lesions and non nephrotic proteinuria. A renal biopsy was performed and was compatible with Henoch-Schönlein nephritis. Serum Amyloid A protein had high levels - 92 mg/L (> 6.8) and a diagnosis of Familial Mediterranean Fever was confirmed by genetic test (homozygote for M694V in MEFV gene). She started colchicine and is doing well, without any further complaints. FMF must be considered in the differential diagnosis of recurrent attacks of fever and abdominal pain in children, even with an atypical presentation (p.e. Protracted Febrile Myalgia Syndrome). Genetic study allows the confirmation of the diagnosis and has prognostic implications.