Chronic recurrent multifocal osteomyelitis in children: diagnostic value of histopathology and microbial testing. Hum Pathol

Universitäts-Kinderklinik und Poliklinik, the Institut für Hygiene und Mikrobiologie, Universität Würzburg, Germany.
Human Pathlogy (Impact Factor: 2.77). 02/1999; 30(1):59-65. DOI: 10.1016/S0046-8177(99)90301-5
Source: PubMed


Chronic recurrent, unifocal or multifocal osteomyelitis (CRMO), an inflammatory disorder of unknown origin, involves different osseous sites and may be associated with palmoplantar pustulosis. Bacterial cultures of affected tissue were reported negative in nearly all cases. Radiological and magnetic resonance imaging features of CRMO have been described, but differential diagnosis remains difficult, including rheumatic diseases, bacterial osteomyelitis, and malignancy. Although definite diagnosis relies on histopathologic confirmation by biopsy, histopathologic criteria have not been defined. Because CRMO may be treated with nonsteroidal antiinflammatory drugs, but not antibiotics, distinguishing CRMO from bacterial osteomyelitis is of major importance. Histopathologic analysis of 12 patients with CRMO indicated a wide variation of reparative changes of bone, but chronic inflammation could not be found at all sites in the same biopsy. The inflammatory infiltrate was mostly scattered, consisting mainly of lymphocytes, plasma cells, histiocytes, and also few neutrophil granulocytes. Immunohistochemistry showed a predominance of CD3(+), CD45RO(+) T-cells, which were mainly CD8(+). In addition, CD20(+) B cells and CD68(+) macrophages were abundant in each biopsy specimen. Mild lymphocytic and granulocytic infiltrates were also detected in three synovial biopsy specimens obtained from adjacent joints. All bacterial and fungal cultures from native biopsy tissues were negative. Amplification of partial-length 16S ribosomal DNA by polymerase chain reaction (PCR) using broad-range eubacterial primers was below the detection limit in all patients. Because histopathologic features alone may not provide conclusive evidence, CRMO should be included in the differential diagnosis of chronic inflammatory bone lesions in children, and the definite diagnosis should be made by the clinical picture, x-ray studies, bone scan, bacterial culture, and histopathologic analysis in a multidisciplinary approach.

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    • "Based on our preliminary data from biopsies collected from 12 CRMO patients [43], we conclude that early inflammation may last for approximately six months, followed by the lymphocytic/plasma cellular phase that may last for several months or years. The fibrosing end-stage may be reached after several years of active bone inflammation [43]. "
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    ABSTRACT: Sterile bone inflammation is the hallmark of autoinflammatory bone disorders, including chronic nonbacterial osteomyelitis (CNO) with its most severe form chronic recurrent multifocal osteomyelitis (CRMO). Autoinflammatory osteopathies are the result of a dysregulated innate immune system, resulting in immune cell infiltration of the bone and subsequent osteoclast differentiation and activation. Interestingly, autoinflammatory bone disorders are associated with inflammation of the skin and/or the intestine. In several monogenic autoinflammatory bone disorders mutations in disease-causing genes have been reported. However, regardless of recent developments, the molecular pathogenesis of CNO/CRMO remains unclear. Here, we discuss the clinical presentation and molecular pathophysiology of human autoinflammatory osteopathies and animal models with special focus on CNO/CRMO. Treatment options in monogenic autoinflammatory bone disorders and CRMO will be illustrated.
    Pediatric Rheumatology 12/2013; 11(1):47. DOI:10.1186/1546-0096-11-47 · 1.61 Impact Factor
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    • "La topographie des lésions inflammatoires peut être uni-ou multifocale. Dans le cas des formes solitaires, l'atteinte se localise plus fréquemment sur le sternum, ou sur le maxillaire inférieur [8] [9]. Dans les formes diffuses, l'atteinte est préférentiellement bilatérale et symétrique. "
    Journal de Radiologie 12/2011; DOI:10.1016/j.jradio.2011.05.016 · 0.57 Impact Factor
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    • "Initially, relying on radiological and gross examination, the list of rare differential diagnoses includes Langerhans cell histiocytoses, Erdheim–Chester disease (ECD), chronic recurrent multifocal osteomyelitis (CRMO), xanthoma, infiltrative storage disorder, malakoplakia, fibrohistiocytic tumor, and metastatic renal cell carcinoma [12–16]. "
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    ABSTRACT: Xanthogranulomatous osteomyelitis is a rare type of inflammatory process which is characterized by composition of immune cell aggregation on histological studies. Delayed-type hypersensitivity reaction of cell-mediated immunity may be implicated in its pathogenesis. Gross and radiological examination can mimic malignancy, and differentiation should be confirmed by histopathological evaluation. We describe the case of a 14-year-old Afghan boy presenting with pain in right shoulder and left leg with prior history of trauma. Fever, limitation in right shoulder range of motion, and tenderness in right shoulder and left thigh were detected following examination. Mild leukocytosis, elevated alkaline phosphatase, and increased erythrocyte sedimentation rate with negative C-reactive protein (CRP) were revealed. X-ray imaging showed mixed density, periosteal reaction, and cortical disruption. Computed tomography (CT) scan revealed lesions involving medulla and cortex, periosteal reaction with soft tissue component, and bone marrow infiltration in right humerus and left fibula. On magnetic resonance imaging (MRI), signal abnormalities in medulla, metaphysis, and diaphysis of the left fibula associated with cortical irregularity and diffuse soft tissue hypersignal areas were demonstrated. Finally, xanthogranulomatous osteomyelitis was confirmed by histological sample. The clinical manifestations and radiographic and laboratory findings of this rare condition are discussed.
    Journal of Orthopaedics and Traumatology 11/2011; 13(4). DOI:10.1007/s10195-011-0165-8
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