Giant and “Granular Melanosomes” in Leopard Syndrome: An Ultrastructural Study
Giant and “Granular Melanosomes” in Leopard Syndrome: An Ultrastructural Study
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豹综合症中的巨型和“颗粒状黑素体”：一项超微结构研究
DOI：
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发表时间：
1976
影响因子：
1.7
通讯作者：
L. Edelstein
中科院分区：
文献类型：
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作者：
J. Bhawan;D. Purtilo;J. A. Riordan;Varun K. Saxena;L. Edelstein
通过电子显微镜观察 LEOPARD 综合征儿童的色素沉着异常情况。在我们的一个病例的雀斑痣中发现了类似于神经纤维瘤病和痣痣的牛奶咖啡斑中所见的巨大黑素体。我们的结果表明，神经纤维瘤病中描述的“球形颗粒黑素体”是与复杂黑色素颗粒发育相关的溶酶体样结构。我们的研究还表明，LEOPARD 综合征的一些角质形成细胞中存在未成熟的黑素体。这一发现与普遍的观念相反，即只有成熟的黑素体才会转移到角质形成细胞。患有LEOPARD综合征的白人皮肤角质形成细胞中存在正常大小和形状的个体黑素体，这表明黑素体的大小和种族差异都不是决定角质形成细胞中黑素体分布的因素。
Electron microscopy of lentigines was performed to study the pigmentation abnormality in two children with LEOPARD syndrome. Giant melanosomes similar to those seen in café‐au‐lait spots of neurofibromatosis and nevus spilus were found in a lentigine from one of our cases. Our results show that „spherical granular melanosomes” described in neurofibromatosis, are lysosomal‐like structures associated with the development of complex melanin granules. Our study also demonstrates that immature melanosomes are present in some keratinocytes of LEOPARD syndrome. This finding is in contrast to the prevailing concept that only mature melanosomes are transferred to keratinocytes. The occurrence of individual melanosomes of normal size and shape in keratinocytes of skin in whites with LEOPARD syndrome, suggests that neither the size of melanosomes, nor the racial differences are the factors determining the distribution of melanosomes in keratinocytes.