Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part three

Résumé fourni par la source

Introduction: Transient periosteal hyperostosis with dysproteinemia, also named Goldbloom syndrome (GS), is a rare pediatric disease characterized by recurrent crisis of bone pain, fever, increased inflammatory markers and dysproteinemia.From 1966 only few cases have been reported in the English literature.Objectives: To better define the clinical and epidemiological features of GS.Methods: We report clinical, laboratory and radiological features of our patients in Table 1, comparing with the English literature.Results: Case 1 was a 9-year-old girl who presented daily crisis of bone pain at the lower limbs, associated with fever spikes, limping and nocturnal awakenings.Physical examination was normal.Laboratory tests showed mild anemia, thrombocytosis, increased inflammatory markers and high antibody levels against streptolysine O and DNase-B (ASO 4280 IU/ml and ADN-B 6310 UI/ml, respectively).Throat swab was positive for group A β-hemolytic streptococcus (GAS).Unusual dysproteinemia, characterized by hypoalbuminemia with increased a1, a2 and g globulinemia, was noted.X-ray evaluation of the lower limbs showed increased bone density at femurs and tibias with signs of periostitis: on STIR sequence MRI these bones presented areas of hyperintense signal.Bone biopsy revealed a thickened periosteum that was strongly adherent to the underlying tissue.Histopathologic study showed signs of chronic inflammation.Steroid treatment was started, leading to a prompt resolution of the clinical picture within few days.Case 2 was a 6-years-old girl who developed, two weeks after an untreated febrile pharyngitis, daily attacks of severe pain at ankles with fever.Joint examination was normal.Throat swab was positive for GAS.In the following weeks, recurrent crisis of bone pain persisted with a severe weight loss.She was hospitalized and laboratory tests showed mild anemia, thrombocytosis and unusual dysproteinemia with hypoalbuminemia and high ND not done

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