| Abstrakt: |
The seventh case of linear scleroderma associated with melorheostosis is presented and the literature reviewed. Melorheostosis is a rare disorder of bone, first described in 1922. It is a peculiar, linear hyperostosis, usually in an extremity, that has been likened to the tallow drippings on the side of a burning candle.The occurrence of these two conditions superimposed in an extremity appears to be particularly disabling. In all seven cases reported the patients were greatly disabled and all but one had shortening of the affected extremity. Four patients had muscle atrophy and three patients, including our own, had chronic dislocation of the patella. The diagnosis was made in childhood in all instances, and in three patients it was present at birth.Both linear scleroderma and melorheostosis are of a linear nature and represent sclerotic pathologic reactions. The coexistence of these two relatively rare disorders in an extremity appears significant. It is suggested that linear scleroderma may represent a primary mesenchymal defect that occasionally spreads into the skeletal tissues. |
