1- Unidade de Infecciologia Pediátrica, Pediatria Médica Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, Lisboa
2- Unidade de Reumatologia Pediátrica, Pediatria Médica Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, Lisboa
3- Unidade de Neuropediatria, Pediatria Médica Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, Lisboa
4- Laboratório de Neuropatologia, Hospital Santa Maria Centro Hospitalar de Lisboa Norte, Lisboa
- reunião internacional, poster
- 35th Annual Meeting of the European Society for Paediatric Infectious Diseases 2017
Introduction: Macrophagic myofasciitis is a rare muscle disease described primarily as a reaction to aluminium-containing vaccines. We present the first description of a case of this entity associated with a post-streptococcal infection.
Case Report: A six years-old boy was admitted with severe myalgia, arthralgia and fever. The pain was symmetrical on the lower limbs, first ankles then knees and in the course of two days also involved shoulders and elbow leaving him unable to walk. The joints were swollen, tender and painful with passive and active movements. He also had rash and red nodular lesions on the anterior face of the legs. There was no history of recent vaccination but he recalled tonsillitis two weeks before. He presented leukocytes 20800/mm3 with 86,3% neutrophils, PCR 80.2mg/L. The creatinine phosphokinase level was normal. The investigation showed only elevated antistreptolysin titers (ASLO 1950UI/mL and antideoxyribonuclease B 749UI/mL) which normalized after 6 weeks He started flucloxaciline and ibuprofen without improvement. The MRI revealed diffuse myosite of various muscles in the lower limb, tenossinuvitis on both feet and arthritis of elbows, knees and ankles. The biopsy of the cutaneous nodular lesions was compatible with leukocytoclastic vasculitis. The muscle biopsy was compatible with macrophagic myofasciitis. Prednisolone was associated with clinical improvement.
Comments Macrophagic myofasciitis occurs mainly in adults, has a more chronic course and is apparently related to aluminium exposure. We described a severe myalgia syndrome with diagnosis of macrophagic myofasciitis with only serological evidence of recent streptococcal infection. This case shows a possible post-streptococcal immune mechanism for this lesion. As other manifestations of the post-streptococcal syndrome, macrophagic myofasciitis appears to have an immune mechanism of lesion an, as such, could be explained in this context
Palavras Chave: leukocytoclastic vasculitis, macrophagic myofasciitis, post-streptococcal syndrome