Holbrook K A, Byers P H, Pinnell S R
Scan Electron Microsc. 1982(Pt 4):1731-44.
Normal, human dermis is a dense, interwoven collagen and elastic matrix organized into papillary and reticular regions. the papillary dermis is a narrow zone beneath the epidermis which includes an even narrower subepidermal connective tissue band beneath the basal lamina. The reticular dermis has superficial intermediate and deep reticular zones. Each region is distinguished by the organization of the fibrous connective tissue. In this review, the structure, composition and function of each region of the normal dermis is surveyed and alterations in the tissue that have been recognized ultrastructurally in skin from patients with inherited connective tissue diseases are discussed. A molecular defect in a connective tissue molecule can be expressed phenotypically at one or more levels of dermal organization and can modify the structure of other matrix components. In some cases, the entire interwoven pattern of the dermis is altered; in others, the dermis retains the interwoven pattern and alterations are expressed as defects in the association of fibrils into fibers and fiber bundles. Abnormalities in the size, packing and relationships of fiber bundles also can occur. These changes involve the collagen matrix primarily. Depletion or exaggeration of elastin, or of the amorphous ground substance can also influence the organization of collagen and of the overall dermal architecture. The alterations described are illustrated with scanning electron micrographs of skin from patients with inherited connective disorders and are discussed in terms of the mechanical abnormalities of the tissue.
正常的人类真皮是一种致密、交织的胶原蛋白和弹性基质，组织成乳头层和网状层。乳头层真皮是表皮下方的一个狭窄区域，包括基膜下方更窄的表皮下结缔组织带。网状层真皮有浅、中、深网状区。每个区域通过纤维结缔组织的组织方式来区分。在这篇综述中，我们考察了正常真皮各区域的结构、组成和功能，并讨论了在遗传性结缔组织病患者皮肤中通过超微结构识别出的组织改变。结缔组织分子中的分子缺陷可在真皮组织的一个或多个层面上表现出表型，并可改变其他基质成分的结构。在某些情况下，真皮的整个交织模式会发生改变；在其他情况下，真皮保留交织模式，改变表现为原纤维聚合成纤维和纤维束过程中的缺陷。纤维束的大小、排列和相互关系也可能出现异常。这些变化主要涉及胶原基质。弹性蛋白或无定形基质的减少或增多也会影响胶原的组织和整个真皮结构。文中用遗传性结缔组织病患者皮肤的扫描电子显微镜照片展示了所描述的改变，并根据组织的机械异常进行了讨论。
