Biochemical and ultrastructural demonstration of elastin accumulation in the skin lesions of the Buschke-Ollendorff syndrome.
Biochemical and ultrastructural demonstration of elastin accumulation in the skin lesions of the Buschke-Ollendorff syndrome.
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Buschke-Ollendorff 综合征皮损中弹性蛋白积累的生化和超微结构证明。
DOI：
10.1111/1523-1747.ep12526121
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发表时间：
1981
期刊：
影响因子：
--
通讯作者：
Murphy,WA
中科院分区：
文献类型：
--
作者：
Uitto,J;SantaCruz,DJ;Starcher,BC;Whyte,MP;Murphy,WA
Buschke-Ollendoff 综合征是皮肤病变、播散性豆状皮肤纤维化和骨质疏松症的一种相关疾病。这种情况以常染色体显性遗传模式遗传。为了阐明皮肤病变的生化性质，我们检查了 12 名 Buschke-Ollendorff 综合征患者，他们代表 2 个不相关的亲属。在组织学上，病变的特征是真皮中存在过量异常宽阔、交错的弹性纤维。用胰弹性蛋白酶纤维消化皮肤切片而不产生碎片。采用放射免疫测定法测量锁链素也证明了皮肤中弹性蛋白的积累。与健康对照的皮肤或病灶附近的单生皮肤相比，皮损的锁链素含量增加了 3 至 7 倍。结果表明，Buschke-Ollendorff综合征的皮损为弹性蛋白型结缔组织痣。这些患者的细胞培养物可以提供一个方便的模型来研究弹性蛋白代谢的控制机制。
The Buschke-Ollendoff syndrome is an association of cutaneous lesions, dermatofibrosis lenticularis disseminata, with osteopoikilosis. This condition is inherited in an autosomal dominant pattern. In order to clarify the biochemical nature of the skin lesions, we have examined 12 patients with the Buschke-Ollendorff syndrome, representing 2 unrelated kindreds. Histologically, the lesions were characterized by excessive amounts of unusually broad, interlacing elastic fibers in the dermis. Digestion of skin sections with pancreatic elastase fibers without fragmention. The accumulation of elastin in the skin was also demonstrated by measurements of desmosine employing a radioimmunoassay. The desmosine content of the skin lesions as increased 3- to 7-fold when compared to the skin either from healthy controls or from univolved skin adjacent to a lesion. The results indicate that the skin lesions of the Buschke-Ollendorff syndrome are connective tissue nevi of the elastin type. Cell cultures form these patients may provide a convenient model to study the control mechanisms involved in elastin metabolism.