Igarashi M, Aizawa H, Tokudome Y, Tagami H
Dermatologica. 1985;170(1):6-11.
Although there is occasionally remarkable accumulation of mucin in the skin lesion of dermatomyositis, no special attention has been paid in regard to mechanisms underlying it. We carried out histochemical and biochemical studies on materials obtained from one case of dermatomyositis, who showed the presence of abundant mucinous material stainable with alcian blue between the collagen bundles throughout the lesional dermis. The major component of the ground substance was identified to be hyaluronic acid. The activity of N-acetyl-beta-glucosaminidase, exoglycosidase that degrades hyaluronic acid in serum of the patient, was equivalent to that of controls. However, heparan sulfate was greatly increased in the urine of the patient in comparison with that of the normal controls in which the major components were chondroitin 4-sulfate and chondroitin 6-sulfate. We think that the increase of hyaluronic acid in the lesional dermis is due to an excess production by the fibroblasts under some immunological stimulation, although its relation to the increased heparan sulfate excretion into the urine of the patient is obscure.
尽管皮肌炎皮肤损害中偶尔会有大量粘蛋白积聚，但对于其潜在机制尚未给予特别关注。我们对1例皮肌炎患者的病变材料进行了组织化学和生化研究，该患者病变真皮层的胶原束之间存在大量可被阿尔辛蓝染色的粘液物质。确定细胞外基质的主要成分是透明质酸。患者血清中降解透明质酸的外糖苷酶N-乙酰-β-葡萄糖苷酶的活性与对照组相当。然而，与主要成分为硫酸软骨素4和硫酸软骨素6的正常对照组相比，该患者尿液中的硫酸乙酰肝素大幅增加。我们认为，病变真皮层中透明质酸的增加是由于在某些免疫刺激下成纤维细胞产生过多，尽管其与患者尿液中硫酸乙酰肝素排泄增加之间的关系尚不清楚。
