Melorheostosis: A Rare Disease
Melorheostosis is a rare nongenetic developmental anomaly first described in 1922 byLeri and Joanny. Its etiology is unknown. Patients present at any age, and both sexes are affectedequally. Onset is usually insidious, with pain, stiffness and limitation of motion at the affected areas.The characteristic radiographic appearance consists of irregular hyperostotic changes of the cortex,generally on one side of the bone, resembling melted wax dripping down one side of a candle. Thereis usually a demarcation line between the affected and normal bone. Dense, sclerotic linear areasare seen mainly in the cortex but also extending into the cancellous bone. Melorheostosis affectsmainly the long bones of the upper and lower limbs, but also the short bones of the hand and footand, rarely, the axial skeleton may be affected. Bone scintigraphy is positive and shows moderatelyincreased uptake of tracer in all three phases. Computed tomography and magnetic resonance imagingcan further characterize the lesion, but rarely contribute to the diagnosis. Treatment of thischronic condition consists of conservative therapies or surgical soft-tissue procedures and even, invery severe cases, amputation. In this case report, a 53 years old male patient with pain and morningstifness in his left hand for 6 months who has been diagnosed as melorheostosis by radiographicappearence is presented.
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