A Pathological Basis for the Cutaneous Papules of Mucopolysaccharidosis II (The Hunter Syndrome)
A Pathological Basis for the Cutaneous Papules of Mucopolysaccharidosis II (The Hunter Syndrome)
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粘多糖贮积症 II（亨特综合症）皮肤丘疹的病理学基础
DOI：
10.1111/j.1600-0560.1977.tb00923.x
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发表时间：
1977
影响因子：
1.7
通讯作者：
R. Freeman
中科院分区：
文献类型：
--
作者：
R. Freeman
用光镜和电子显微镜技术对2例Hunter综合征(粘多糖症II)患者的皮肤丘疹与正常皮肤进行了比较。正常皮肤和丘疹皮肤的大多数纤维细胞含有异染的细胞质和特征性的空泡，只有丘疹有异染物质的细胞外堆积和空泡的融合和破裂。酒精固定可以更好地保存粘膜物质。
Cutaneous papules of two patients with the Hunter syndrome (mucopolysaccharidosis II) were compared with their normal appearing skin by light and electron microscopic techniques. While most fibrocytes of normal and papular skin contained metachromatic cytoplasmic material and characteristic vacuoles, only the papules had extracellular accumulation of metachromatic material and evidence of coalescence and rupture of vacuoles. Alcohol fixation provides better preservation of the mucosubstances.