Winik B C, Boente M C, Asial R
Laboratorio de Microscopía electrónica del Noroeste Argentino, CONICET, Tucumán, Argentina.
Am J Dermatopathol. 1998 Aug;20(4):373-8. doi: 10.1097/00000372-199808000-00009.
Juvenile hyaline fibromatosis is a multisystemic disorder characterized by a triad of cephalic fibrous outgrowths, gingival hyperplasia, and flexion contractures. The aim of this study was to find new ultrastructural features that could be useful for differentiating this entity from other types of fibromatosis. Mucosal lesions processed for light and electron microscopy by routine techniques showed hyperactive-appearing spindle-shaped fibroblasts and dysplastic mesenchymal cells. Dilated rough endoplasmic reticulum, prominent Golgi complexes, and multivesicular bodies as well as single membrane vesicles filled with fibrillogranular material were seen within the cytoplasm of dysplastic mesenchymal cells. Many fibrillogranular vesicles contained smaller vesicles. There were also invaginations of the cell membrane that contained fibrillogranular material similar to that seen in the single membrane vesicles, suggesting engulfment of an extracellular substance. The stroma contained both normal and serrated collagen fibrils, microfibrils, and two types of fibrillogranular material, one of them with a characteristic banding pattern. Our clinical and histopathologic findings resemble those previously described in cases of infantile systemic hyalinosis and juvenile hyaline fibromatosis. So many features of these two conditions overlap that it is difficult not to consider them as parts of a spectrum of the same disorder.
青少年透明纤维瘤病是一种多系统疾病，其特征为三联征：头部纤维性赘生物、牙龈增生和屈曲挛缩。本研究的目的是寻找新的超微结构特征，以有助于将该实体与其他类型的纤维瘤病区分开来。通过常规技术处理用于光镜和电镜检查的黏膜病变显示出外观活跃的梭形成纤维细胞和发育异常的间充质细胞。在发育异常的间充质细胞的细胞质内可见扩张的粗面内质网、突出的高尔基体复合体、多囊泡体以及充满纤维颗粒物质的单膜小泡。许多纤维颗粒小泡含有更小的小泡。细胞膜也有内陷，其中含有与单膜小泡中所见相似的纤维颗粒物质，提示吞噬了细胞外物质。间质包含正常和锯齿状的胶原纤维、微原纤维以及两种类型的纤维颗粒物质，其中一种具有特征性的条纹图案。我们的临床和组织病理学发现与先前报道的婴儿全身性透明变性和青少年透明纤维瘤病病例相似。这两种情况的许多特征重叠，以至于很难不将它们视为同一疾病谱的一部分。
