Occlusive Nonvasculitic Vasculopathy

Abstract

We review the most characteristic clinical and histopathologic findings of the cutaneous manifestations of the occlusive nonvasculitic vasculopathic disorders. Clinically, most of these conditions are characterized by retiform purpura. Histopathologic findings consist of occlusion of the vessel lumina with no vasculitis. Different disorders may produce nonvasculitic occlusive vasculopathy in cutaneous blood and lymphatic vessels, including embolization due to cholesterol and oxalate emboli, cutaneous intravascular metastasis from visceral malignancies, atrial myxomas, intravascular angiosarcoma, intralymphatic histiocytosis, intravascular lymphomas, endocarditis, crystal globulin vasculopathy, hypereosinophilic syndrome, and foreign material. Other times, the occlusive disorder is due to platelet pugging, including heparin necrosis, thrombocytosis secondary to myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, and thrombotic thrombocytopenic purpura. Occlusive vasculopathy may also appear in cold-related gelling agglutination, like that occurring in cryofibrinogenemia, cryoglobulinemia, cold agglutinin syndrome, and crystalglobulinemia. Microorganisms may also occlude the vessels lumina and this is especially frequent in ecthyma gangrenosum, opportunistic fungi as aspergillosis or fusariosis, Lucio phenomenon of lepromatous leprosy and disseminated strongyloidiasis. Systemic coagulopathies due to defects of C and S proteins, coumarin/warfarin-induced skin necrosis, disseminated intravascular coagulation, and antiphospholipid antibody/lupus anticoagulant syndrome may also result in occlusive nonvasculitic vasculopathy. Finally, vascular coagulopathies such as Sneddon syndrome, livedoid vasculopathy, and atrophic papulosis may also cause occlusion of the vessels of the dermis and/or subcutis. Histopathologic study of occlusive vasculopathic lesions is the first step to achieve an accurate diagnosis, and they should be correlated with clinical history, physical examination, and laboratory findings to reach a final diagnosis.

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

“…Although many aspects of its pathogenesis have not been clearly delineated, we regard livedo vasculopathy as a characteristic clinically and histopathologically defined process [24,25], caused by a variety of coagulation disorders [11,22], (such as AT-III deficiency, protein-C and protein-S deficiency, APC resistance, cryoglobulinemia, cold agglutinin disease, cryofibrinogenemia), even though one cannot always detect or define such a defect. Clinical appearance and histopathological findings (Table 2) are characteristically independent of etiology and pathomechanism.…”

“…Thrombi can also be observed, but they are a secondary phenomenon caused by damage to the vessel wall. Occluding vasculopathies [11] can be caused by proliferations such as malignancies, by embolization of different materials (cholesterol, oxalate, microorganisms) or most commonly by coagulopathies with occlusion by thrombi. Coagulopathies differ from vasculitides in their initiation by abnormal blood coagulation.…”

Ecthyma gangrenosum caused byCitrobacter freundii

“…Classically, EG is characterised by bacterial invasion of the venule which then leads to secondary arteriole thrombosis, tissue oedema and separation of the epidermis. This results in formation of characteristic lesions associated with EG—vesicles or blisters surrounded by an erythematous or violaceous halo that can progress to haemorrhagic bullae with black necrotic centres 4. Usual skin sites of infection include the buttocks and lower extremities (66%), although a significant number of cases have resulted in involvement with the face, head and neck (8%) 5…”

Barcodeleser – ein algorithmischer Ansatz für okkludierende kutane Vaskulopathien? Teil I: Vaskulopathien kleiner Gefäße

“…Obwohl viele Aspekte ihrer Pathogenese noch nicht eindeutig beschrieben sind, halten wir die Livedovaskulopathie für einen charakteristischen, klinisch und histopathologisch definierten Prozess , der durch eine Vielzahl von Gerinnungsstörungen verursacht wird (beispielsweise AT‐III‐Defizienz, Protein‐C‐ und Protein‐S‐Defizienz, APC‐Resistenz, Kryoglobulinämie, Kälteagglutininkrankheit, Kryofibrinogenämie), selbst wenn ein derartiger Defekt nicht immer erkannt oder definiert werden kann. Das klinische Erscheinungsbild und die histopathologischen Befunde (Tabelle ) sind in der Regel unabhängig von Ätiologie und Pathomechanismus.…”

“…Thromben können zwar ebenfalls auftreten, sind jedoch ein sekundäres Phänomen, das durch die Schädigung der Gefäßwand verursacht wird. Okkludierende Vaskulopathien können durch eine Proliferation wie bei malignen Erkrankungen, durch Embolisation verschiedener Materialien (Cholesterin, Oxalat, Mikroorganismen) oder, am häufigsten, durch Koagulopathien mit einem Verschluss durch Thromben verursacht werden. Koagulopathien unterscheiden sich von den Vaskulitiden dadurch, dass ihre Entstehung auf einer abnormalen Blutgerinnung beruht.…”