Akimoto S, Ishikawa O, Yokoyama Y, Amano H, Miyachi Y
Department of Dermatology, Gunma University, School of Medicine, Maebashi, Japan.
Acta Derm Venereol. 1996 Mar;76(2):141-3. doi: 10.2340/0001555576141143.
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.
我们报告了一名69岁患有严重泛发性硬斑病的男性患者，其皮肤受累面积超过80%，但内脏未受影响。我们对硬化性皮损的中央和周边以及临床上未受累皮肤进行了组织学检查，并分析了构成软骨素酶可消化糖胺聚糖的皮肤二糖。在硬化性皮损的中央和周边部位，整个真皮和皮下组织均可见硬化性纤维化以及与硬斑病相符的密集血管周围细胞浸润。此外，还观察到各种血管变化，如内皮细胞肿胀、基底膜增厚以及脂肪小叶内血管腔阻塞。在临床上未受累的皮肤中，间质水肿明显，并伴有轻微的血管周围细胞浸润。在二糖分析中，不仅在硬化性皮损中，而且在临床上未受累的皮肤中（尽管不太明显），均发现硫酸皮肤素的主要二糖单位δDi-4S(DS)、硫酸软骨素的主要二糖单位δDi-6S和δDi-6S的量增加，而源自透明质酸的δDi-HA减少。这些改变与系统性硬化症一致，提示严重形式的泛发性硬斑病与系统性硬化症之间存在密切关系。
