Wollina U, Reuter A, Schaarschmidt H, Müller E, Maak B, Schmidt U
Klinik und Poliklinik für Hautkrankheiten, Friedrich-Schiller-Universität, Jena.
Hautarzt. 1992 Jul;43(7):453-7.
A case report on a 6-year-old boy suffering from the extremely rare Hutchinson-Gilford syndrome (progeria) is presented. The results of histopathological and immunohistological examination of the scar-like skin lesions are reported. Subcutaneous amorphous nodules were eosinophilic, PAS- und elastica-negative und remained unstained with antibodies against collagen type IV, vimentin, and collagenase. The dense perivascular infiltration consisted of CD4+, CD8-, alpha-1-antichymotrypsin-, MAC 387-, and some vimentin-positive cells. Perinodular blood vessels were more abundant and had a thickened wall. Collagen bundles were swollen. The epidermis appeared atrophic with focal basal cell degeneration.
本文报告了一例患有极为罕见的哈钦森-吉尔福德综合征（早老症）的6岁男孩病例。报告了瘢痕样皮肤病变的组织病理学和免疫组织学检查结果。皮下无定形结节呈嗜酸性，过碘酸雪夫反应（PAS）和弹性组织染色阴性，并且用抗IV型胶原、波形蛋白和胶原酶的抗体染色后不着色。密集的血管周围浸润由CD4 +、CD8 -、α-1-抗胰凝乳蛋白酶、MAC 387以及一些波形蛋白阳性细胞组成。结节周围的血管更丰富且管壁增厚。胶原束肿胀。表皮出现萎缩并伴有局灶性基底细胞变性。
