Takata M, Imai T, Hirone T
Department of Dermatology, Kanazawa University School of Medicine, Japan.
Am J Dermatopathol. 1994 Feb;16(1):37-43. doi: 10.1097/00000372-199402000-00008.
Cutaneous neurofibromas consist of heterogenous cell populations including Schwann cells, perineurial cells, and fibroblastlike cells. However, the histogenesis of neurofibromas, particularly the origin and nature of the fibroblastlike cells, is still controversial. Recently, cells containing blood coagulation factor XIIIa have been reported in cutaneous neurofibromas, although their identity is uncertain. In this report, by the combination of double immunohistochemical staining and immunoelectron microscopy, we demonstrate that factor-XIIIa-positive cells are distinct from Schwann cells, perineurial cells, endothelial cells, mast cells, and conventional macrophages, but correspond to the fibroblastlike cells in cutaneous neurofibromas. Such factor-XIIIa-positive cells in cutaneous neurofibromas, however, differ from conventional fibroblasts in the strong expression of HLA-DR antigen and lack of prolyl 4-hydroxylase. Similarly, so-called endoneurial fibroblasts and, occasionally, connective tissue cells within perineurium and epineurium in normal peripheral nerve fibers express factor XIIIa as well as HLA-DR antigen. The results suggest that fibroblastlike cells in cutaneous neurofibromas are probably derived from factor-XIIIa- and HLA-DR antigen-positive connective tissue cells in peripheral nerves. The role of such factor-XIIIa-positive cells in the growth and development of cutaneous neurofibromas is discussed.
皮肤神经纤维瘤由包括施万细胞、神经束膜细胞和成纤维样细胞在内的异质细胞群组成。然而，神经纤维瘤的组织发生，尤其是成纤维样细胞的起源和性质，仍然存在争议。最近，在皮肤神经纤维瘤中报道了含有凝血因子 XIIIa 的细胞，尽管它们的身份尚不确定。在本报告中，通过双重免疫组织化学染色和免疫电子显微镜相结合的方法，我们证明因子 XIIIa 阳性细胞与施万细胞、神经束膜细胞、内皮细胞、肥大细胞和传统巨噬细胞不同，但与皮肤神经纤维瘤中的成纤维样细胞相对应。然而，皮肤神经纤维瘤中的这种因子 XIIIa 阳性细胞与传统成纤维细胞不同，其 HLA-DR 抗原表达强烈且缺乏脯氨酰 4-羟化酶。同样，正常周围神经纤维中的所谓神经内膜成纤维细胞，以及偶尔神经束膜和神经外膜内的结缔组织细胞也表达因子 XIIIa 和 HLA-DR 抗原。结果表明，皮肤神经纤维瘤中的成纤维样细胞可能来源于周围神经中因子 XIIIa 和 HLA-DR 抗原阳性的结缔组织细胞。本文讨论了这种因子 XIIIa 阳性细胞在皮肤神经纤维瘤生长和发育中的作用。
