RESUMO
Los síndromes autoinflamatorios son un grupo de enfermedades caracterizadas por episodios espontáneos, recurrentes o persistentes de inflamación multisistémica. En ellos no se evidencia una etiología infecciosa, neoplásica o autoinmune. Están causadas por alteraciones de la inmunidad innata, lo que ocasiona una desregulación del sistema inflamatorio a nivel del inflamosoma. Estos síndromes se subdividen en dos grandes grupos los síndromes hereditarios de fiebre periódica y las enfermedades autoinflamatorias persistentes, dentro de este último se ubican las enfermedades inflamatorias óseas donde se incluye la osteomielitis multifocal crónica recurrente. Presentamos un caso de una niña de 9 años que ingresa en nuestro hospital por un síndrome febril prolongado y después de múltiples investigaciones se concluye como una osteomielitis multifocal crónica recurrente.
Autoinflammatory syndromes are a group of diseases characterized by spontaneous, recurrent or persistent episodes of multisystem inflammation. They do not show an infectious, neoplastic or autoimmune etiology. They are caused by alterations of the innate immunity, which causes a dysregulation of the inflammatory system at the level of the inflammasome. These syndromes are subdivided into two major groups, the hereditary syndromes of periodic fever and the persistent autoinflammatory diseases; within the latter are the inflammatory bone diseases which chronic recurrent multifocal osteomyelitis. We present a case of a 9-year-old girl who enters our hospital due to a prolonged febrile syndrome and after multiple investigations, it concludes as a Chronic Recurrent Multifocal Osteomyelitis.
RESUMO
Background and Objectives: Chronic recurrent mutlifocal osteomyelitis (CRMO) is an extremely rare skeletal disorder in the younger population. It presents with multifocal bony lesions that often mimic more sinister diagnoses such as neoplasm. The cause of this condition remains unknown and there is limited evidence on effective treatment.Presentation of Case: A 9-year-old girl presented to our institution with non-traumatic onset of left leg pain. After failed conservative management, radiographs and MRI were obtained exhibiting a bony lesion of the proximal tibia resembling osteomyelitis. The patient was non-responsive to antibiotics, so corticotomy and drainage was done in which only blood came out of the lesion, no pus was seen. Patient improved dramatically but again she developed similar symptoms and signs on right leg. Biopsy from left leg suggested no significant findings.Discussion: Chronic recurrent multifocal osteomyelitis presents in patients with periodic fevers, bone pain and bone lesions that can develop anywhere in the body. This is a rare disease, which has been found to affect more girls than boys.Conclusion: CRMO should be considered as a differential diagnosis for chronic bone pain with affinity for the long bones of the lower extremity in children and adolescents.