Bar code reader – an algorithmic approach to cutaneous occluding vasculopathies? Part I: small vessel vasculopathies

Abstract

SummaryAimsThe classifications of occluding vasculopathies may present some difficulties. Firstly, classifications may follow different principles, e.g. clinicopathological findings, etiology or pathomechanism. Secondly, authors sometimes do not distinguish between vasculitis and vasculopathy. Thirdly, vasculopathies are often systemic diseases. Organ‐specific variations make morphologic findings difficult to compare. Moreover, subtle changes may be recognized in the skin, but be invisible in other organs. Our aim was to use the skin and subcutis as tools and clinicopathological correlation as the basic process for classification.Methods and resultsIn the first step, we differentiate between small and medium vessel occluding vasculopathies in the skin, and focus in this part on small vessel occluding vasculopathies. In the second step, we differentiate among subtypes of small vessels. In the final step, we differentiate according to the time point of the coagulation/reorganization process and the involved inflammatory cells/stromal features. Applying the same procedure to the various entities and visualizing the findings with bar codes makes the similarities and differences more apparent, both clinically and with histopathology.ConclusionOccluding vasculopathies are often not separate entities, but reaction patterns and epiphenomena. Distinguishing them from vasculitides is crucial because of differences in pathogenesis, therapeutic approach and prognosis.

Monoclonal gammopathy with cutaneous significance treated successfully with rituximab

“…The skin manifestations result from vascular deposition of fragments of the immunoglobulins. This can lead to both occluding vasculopathy and inflammation of the vessels (vasculitis) [4,5]. In our two patients MGUS was diagnosed based on the M protein in the electrophoresis and the presence of the light chains in the immunohistological examination.…”

Generalisierte symmetrische Teleangiektasien

“…In einigen Fällen werden intravaskuläre Mikrothromben gefunden [8]. Diese scheinen jedoch eher ein Epiphänomen als die Ursache der CCV zu sein (für eine algorithmische Herangehensweise an kutane okkludierende Vaskulopathien, die oft keine eigene Entität, sondern ein Reaktionsmuster oder ein Epiphänomen darstellen, siehe [9]).…”

Cutaneous vasculitis

“…In the contrary, one must separate occluding vasculopathies. 3,4 Here, the pathologic process mostly starts in the blood with variable occlusion of blood vessels due to hypercoagulability, in particular by thrombi and emboli. The distinction between the primary event being vasculitis or occluding coagulopathy has important therapeutic consequences: classical vasculitides benefit from immunosuppressive therapies; microorganism-associated vasculitides need specific antimicrobial agents; and coagulopathies require anticoagulant therapy.…”