Clinically, the skin changes observed in a 34-year-old woman were suggestive of the reticular erythematosis mucinosis (REM) syndrome. On palpation, however, the changes suggested the plaque-type REM syndrome. Histologically, broad, well-delineated cell-rich bundles were found with abundant alcian-blue-positive material, together with a lymphocytic infiltrate. In such areas showing close contact with the lymphocytic infiltrate, stellate cells with delicate blue processes and protoplasmic granula predominated. Areas preferentially affected with deposits of mucopolysaccharides in the REM syndrome and its variants were not affected in the case presented. The histogenesis of this hitherto undescribed cutaneous mucinosis is discussed.
临床上,一名34岁女性身上观察到的皮肤变化提示为网状红斑性粘蛋白沉积症(REM)综合征。然而,触诊时这些变化提示为斑块型REM综合征。组织学上,发现有宽阔、界限清晰且细胞丰富的束状物,伴有大量阿尔辛蓝阳性物质,以及淋巴细胞浸润。在与淋巴细胞浸润紧密接触的此类区域,以具有纤细蓝色突起和原生质颗粒的星状细胞为主。REM综合征及其变体中优先受粘多糖沉积影响的区域在该病例中未受影响。本文讨论了这种迄今未描述过的皮肤粘蛋白沉积症的组织发生。