Department of Dermatology, Venereology and Allergology, Medical University Innsbruck, Innsbruck, Austria.
Department of Pathology, Medical University Innsbruck, Innsbruck, Austria.
J Dtsch Dermatol Ges. 2019 Nov;17(11):1115-1128. doi: 10.1111/ddg.13973.
Classifications of occluding vasculopathies (except vasculitis [1]) may exhibit some difficulties. Firstly, classifications may follow different principles, e.g. clinicopathologic findings, etiology or pathogenesis. Secondly, authors may not distinguish between vasculitis and occluding vasculopathies. Thirdly, occluding vasculopathies are systemic diseases. Organ-specific variations make morphologic findings difficult to compare. Moreover, subtle changes are recognized in the skin, but may be invisible in other organs. Our aim was to use the skin and subcutis as a tool and clinicopathological correlation as the basic process for classification.
We first differentiate in the skin between small and medium vessel occluding vasculopathies. Here we focus on medium vessel-occluding vasculopathies. In the second step we differentiate the vessel subtypes. In the final step, we differentiate according to the time point of the coagulation/reorganization process and the involved inflammatory cells/stromal features. By applying the same procedure to the various entities and visualizing the findings in the style of bar codes, the overlaps and differences in the clinical picture as well as the histopathology become more apparent.
Occluding vasculopathies are often not separate entities, but reaction patterns and epiphenomena. Distinguishing them from vasculitides is crucial because of the differences in pathogenesis, therapeutic approach and prognosis.
除血管炎[1]外,闭塞性血管病(occluding vasculopathies)的分类可能存在一些困难。首先,分类可能遵循不同的原则,例如临床病理发现、病因或发病机制。其次,作者可能无法区分血管炎和闭塞性血管病。第三,闭塞性血管病是系统性疾病。器官特异性变化使得形态学发现难以比较。此外,皮肤中的细微变化可以被识别,但在其他器官中可能不可见。我们的目的是使用皮肤和皮下组织作为工具,并将临床病理相关性作为分类的基本过程。
我们首先在皮肤中区分小血管和中血管闭塞性血管病。在这里,我们重点关注中血管闭塞性血管病。在第二步中,我们区分血管亚型。在最后一步中,我们根据凝血/重组过程的时间点以及涉及的炎症细胞/基质特征进行区分。通过对各种实体应用相同的程序,并以条码的形式可视化发现,临床特征和组织病理学的重叠和差异变得更加明显。
闭塞性血管病通常不是单独的实体,而是反应模式和伴随现象。将它们与血管炎区分开来至关重要,因为它们在发病机制、治疗方法和预后方面存在差异。