Archive for January, 2008
What is Melorheostosis?
Melorheostosis is a rare, non-hereditary bone disease which affects both sexes. It affects both bone and soft tissue, and while benign, can result in severe functional limitation, extensive pain, and significant deformity. It is usually apparent in early childhood and even in the first few days of life. About 50% of persons affected will develop the symptoms by the time they are 20 years of age. On x-rays, the disease’s sclerotic bone lesions looks like wax dripping down the side of a candle. Hence its name; which is derived from the greek melos “limb” and rhein “to flow”. With an estimated incidence of 0.9 cases per million persons, it can be calculated that there are approximately 1,000,000 to 1,500,000 persons with meloreostosis currently alive. However, only about 300 cases have been reported. Patients with meloreostosis may also have associated cutaneous and soft tissue lesions such as vascular malformations, neurofibromatosis, hemangioma, arterial aneurysms, linear scleroderma, tuberous sclerosis, hemangiomas, and focal subcutaneous fibrosis. Adults generally complain of pain, joint stiffness, and progressive deformity. In children the condition affects mainly the bones of the extremities and pelvis, and may result in limb length inequality, deformity, or joint contractures. Joint contractures may be accompanied by extraosseous bone formation. The ...
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