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Linear scleroderma and melorheostosis

Alan Siegel, MD and Hadyn Williams, MD ^*,

Department of Radiology, Division of Nuclear Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA ^* Department of Nuclear Medicine, Veterans Administration Medical Center, Philadelphia, Pennsylvania, USA

This excerpt was created in the absence of an abstract.

Melorheostosis is an uncommon sclerosing bone dysplasia, which has been associated with other mesenchymal abnormalities. We present a patient with linear scleroderma who has radiographic and scintigraphic confirmation of melorheostosis. Bone scintigraphy in a patient with linear scleroderma and melorheostosis has not, to the best of our knowledge, been previously described.

A 65-year-old woman was admitted for treatment of bilateral plantar fasciitis.

Diagnosis of melorheostosis and linear scleroderma of the left leg was made in 1945 when the patient was 23 years of age. The disease process had reportedly been present since child-hood. Owing to pain and limitation of movement in the left leg, she underwent a fasciotomy in the 1950s. Associated abnormalities include oesophageal dysmotility and Raynaud's phenomenon, for which she underwent a lumbar sympathectomy in 1953.

Examination of the left leg revealed skin thickening on the medial aspect from the knee to the lower leg. Multiple scars were present, secondary to previous surgery.

Radiographs revealed cortical hyperostosis of the medial femoral condyle, which apparently crossed the joint space, extended into the medial aspects of the proximal tibial plateau and continued down the entire length of the medial tibial diaphysis. There was narrowing of the medial compartment of the left knee (Fig. 1).

Bone scintigraphy, performed 4 h following intravenous administration of 925 MBq (25 mCi) of technetium-99m methylene diphosphonate, demonstrated increased activity in the medial left femoral condyle, crossing the joint space into the medial aspect of the tibial plateau.

Key Words: Melorheostosis • Scleroderma • Hyperostosis

Current address: Nuclear Medicine Department, Georgia Baptist Medical Center, Atlanta, Georgia, USA.

Received for publication March 5, 1991. Revision received August 6, 1991. Accepted for publication September 4, 1991.

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