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A new case of this unusual entity is reported. The patient, male aged 43 has an hypertrophy of the first and second left fingers. Radiographic examination shows bony overgrowth and marked soft tissue thickening. Arteriography did not show any evidence of angioma or arteriovenous fistula. Histologic examination shows deposits of fat in soft tissue thickening. Macrodystrophia lipomatosa is a rare disease, usually confined to one or two digits of the hand, and sometimes of the foot. A dysembryoplasic aetiology could be sustained, and this entity is very close from others bone or soft-tissue hamartomas.
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