“ Rare, spontaneous mesodermal disease of unknown aetiology, often asymptomatic initially. It follows a slow course in adults and a more rap...
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Rare, spontaneous mesodermal disease of unknown aetiology, often asymptomatic initially. It follows a slow course in adults and a more rapid course in children. It mostly affects one or more long bones of the upper or lower limbs, may be in a dermatome distribution and may cause thickening/fibrosis of the overlying skin.
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• Progressive cortical hyperostosis along one side of the affected bone in a ‘sclerotome’ (skeletal zones supplied by individual spinal sensory nerves).
• The appearance is of dripping candle wax, centred on the diaphysis.
• Can cross joints, leading to flexion contractures or fusion.
• Look for genu varus, genu valgus and leg length discrepancy.
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