Kretzschmar L, Hamm H, John S M, Bröcker E B
Universitäts-Hautklinik Münster.
Hautarzt. 1992 Oct;43(10):640-4.
Elastosis perforans serpiginosa (EPS) is a rare entity belonging to the group of primary perforating dermatoses. A 13-year-old male patient with Down's syndrome developed reddish hyperkeratotic papules in a serpiginous and ellipsoid configuration on the face. Histological examination revealed transepidermal elimination of thick coarse elastic fibres from the papillary dermis. The dermal infiltrate showed an immunohistological pattern consistent with an acute cell-mediated immune response. It consisted mainly of activated T-lymphocytes, with a predominance of CD4-positive cells. Considerable numbers of CD1-positive cells were also present. Inflammatory macrophages of the 27E10 phenotype were found in considerable numbers, whereas 25F9-positive resident macrophages were almost completely absent. The role of a cell-mediated immune response in the mechanism of transepithelial elimination is discussed.
匐行性穿通性弹力纤维病（EPS）是一种罕见的原发性穿通性皮肤病。一名13岁患有唐氏综合征的男性患者面部出现呈匐行状和椭圆形的微红角化过度丘疹。组织学检查显示从乳头真皮层有粗大弹性纤维的经表皮清除。真皮浸润呈现出与急性细胞介导免疫反应一致的免疫组织学模式。主要由活化的T淋巴细胞组成，以CD4阳性细胞为主。也存在相当数量的CD1阳性细胞。发现大量27E10表型的炎性巨噬细胞，而25F9阳性的常驻巨噬细胞几乎完全缺失。本文讨论了细胞介导免疫反应在经上皮清除机制中的作用。
