Morgan M B, Truitt C A, Taira J, Somach S, Pitha J V, Everett M A
Department of Pathology: University of South Florida Health Science Center, James A. Haley Veteran's Hospital, Tampa, USA.
Am J Dermatopathol. 1998 Apr;20(2):147-54. doi: 10.1097/00000372-199804000-00008.
Despite detailed microscopic descriptions and clinical observation, little is known regarding the pathogenesis of the perforating disorders of skin, which have traditionally been subdivided into numerous microscopic entities associated with various clinical settings. An increasing body of evidence now suggests that the perforating disorders of skin are akin, and may constitute an expanded single pathologic entity. Each of the classic perforating disorders of skin, including elastosis perforans serpiginosa, perforating folliculitis, reactive perforating collagenosis, Kyrle's disease, and perforating disorder of uremia, have been shown to extrude collagen, elastin, and related extracellular matrix components through the epidermis. Considering a shared pathogenic mechanism among these entities, we explored the possible role of the extracellular matrix, in particular fibronectin, in perforating disorders of skin. Using immunohistochemical and serum determinations of extracellular matrix constituents, including fibronectin, collagen type IV, laminin, and tenascin, we showed consistent serum elevation and/or deposition of fibronectin, in each case, without a commensurate increase in laminin, collagen type IV, and tenascin. We propose that elevated serum and tissue concentrations of fibronectin may be responsible for inciting, in a physiologically aberrant manner, increased epithelial migration and proliferation culminating in perforation.
尽管有详细的微观描述和临床观察，但对于皮肤穿通性疾病的发病机制仍知之甚少，这些疾病传统上被细分为与各种临床情况相关的众多微观实体。现在越来越多的证据表明，皮肤穿通性疾病具有相似性，可能构成一个扩展的单一病理实体。每种经典的皮肤穿通性疾病，包括匐行性穿通性弹力纤维病、穿通性毛囊炎、反应性穿通性胶原病、 Kyrle病和尿毒症性穿通性疾病，都已被证明可通过表皮排出胶原蛋白、弹性蛋白和相关的细胞外基质成分。考虑到这些实体之间存在共同的致病机制，我们探讨了细胞外基质，特别是纤连蛋白，在皮肤穿通性疾病中的可能作用。通过免疫组织化学和对包括纤连蛋白、IV型胶原、层粘连蛋白和腱生蛋白在内的细胞外基质成分进行血清检测，我们发现每种情况下纤连蛋白的血清水平均持续升高和/或沉积，而层粘连蛋白、IV型胶原和腱生蛋白却没有相应增加。我们认为，血清和组织中纤连蛋白浓度升高可能以生理异常的方式，促使上皮细胞迁移和增殖增加，最终导致穿孔。
