An ultrastructural study of the reactive type of elastosis perforans serpiginosa.
An ultrastructural study of the reactive type of elastosis perforans serpiginosa.
复制标题
反应型穿孔弹力纤维的超微结构研究。
DOI：
10.1001/archderm.1987.01660330034007
复制
发表时间：
1987
影响因子：
--
通讯作者：
C. Lichtig
中科院分区：
文献类型：
--
作者：
R. Bergman;R. Friedman‐Birnbaum;R. Ludatscher;C. Lichtig
致编辑。-自首次描述穿孔丝状纤维弹性症（EPS）以来，一直认为这是一种由多种异常弹性纤维引起的现象。目前认为至少有三种病因类型涉及，即特发性、反应性和青霉胺诱导。先前的超微结构研究表明，特发性型的异常弹性纤维在结构上与青霉胺诱导型不同。1,3-6反应型与不同的先天性疾病有关，主要与结缔组织有关，如唐氏综合征、成骨不全症、马凡氏综合征、弹性假性黄瘤、ehers - danlos综合征（EDS）等，但研究尚未广泛。它是否是EPS的一种独特的结构形式还有待证实。我们对两例反应型EPS患者进行了超微结构研究。病例报告。案例1。-一名15岁女孩，表现为成骨不全，Ia型，
To the Editor.— Since elastosis perforans serpiginosa (EPS) was first described, it has been suggested that it is a phenomenon caused by a variety of abnormal elastic fibers. At least three etiologic types are now considered to be involved, ie, the idiopathic, the reactive, and the penicillamine-induced. 1-3 Previous ultrastructural investigations showed that the abnormal elastic fibers in the idiopathic type differ in structure from those of the penicillamine-induced type. 1,3-6 The reactive type, which is associated with different congenital disorders, mostly of the connective tissue, such as Down's syndrome, osteogenesis imperfecta, Marfan's syndrome, pseudoxanthoma elasticum, and Ehlers-Danlos syndrome (EDS), has not been as extensively studied. 7 It remains to be demonstrated whether it is a unique structural form of EPS. We performed an ultrastructural study in two patients with the reactive type of EPS. Report of Cases.—Case 1.— A 15-year-old girl presented with osteogenesis imperfecta, type Ia,