Generalized morphea with vascular involvement. A case report and disaccharide analysis of the skin glycosaminoglycans.

We report a 69-year-old man with severe generalized morphea, who showed over 80% of skin involvement, while the internal organs were not affected. We performed histological examinations and analysis of skin disaccharides constituting chondroitinase-digestible glycosaminoglycans in the center and periphery of the sclerotic lesions and the clinically uninvolved skin. In both the central and peripheral parts of the sclerotic lesions, sclerotic fibrosis and a dense perivascular cell infiltration, consistent with morphea, were seen in the entire dermis and subcutis. Furthermore, various vascular changes were observed, such as endothelial cell swell, thickened basement membrane and obstruction of vascular lumen in the fat lobules. In the clinically uninvolved skin, interstitial edema was prominent along with a slight perivascular cell infiltration. On disaccharide analysis, the increase in the amount of delta Di-4S(DS), the main disaccharide unit of dermatan sulphate, delta Di-6S and delta Di-6S, the main disaccharide units of chondroitin sulphate, and the decrease in delta Di-HA, which is derived from hyaluronate, were found not only in the sclerotic lesions but also in the clinically uninvolved skin, though less prominent. These alterations were consistent with systemic sclerosis, suggesting a close relationship between severe forms of generalized morphea and systemic sclerosis.