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Occlusive Nonvasculitic Vasculopathy

Llamas-Velasco, Mar MD*; Alegría, Victoria MD; Santos-Briz, Ángel MD; Cerroni, Lorenzo MD§; Kutzner, Heinz MD; Requena, Luis MD

The American Journal of Dermatopathology: September 2017 - Volume 39 - Issue 9 - p 637–662
doi: 10.1097/DAD.0000000000000766
CME Article
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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.

*Associate Staff of the Department of Dermatology, Department of Dermatology, Hospital Universitario de la Princesa, Universidad Autónoma, Madrid, Spain;

Associate Staff of the Department of Dermatology, Department of Dermatology, Fundación Jiménez Diaz, Universidad Autónoma, Madrid, Spain;

Director of Dermatopathology, Department of Dermatology, Hospital Clínico Universitario, Salamanca, Spain;

§Director of Dermatopathology, Dermatopathology Research Unit, Medical University of Graz, Graz, Austria;

Director of Dermatopathology, Dermatopathologie Laboratory, Friedrichschafen, Germany; and

Chairman of the Department of Dermatology, Department of Dermatology, Hospital Clínico Universitario, Salamanca, Spain.

Reprints: Luis Requena, MD, Department of Dermatology, Fundación Jiménez Díaz, Avda. Reyes Católicos 2, 28040-Madrid, Spain (e-mail: lrequena@fjd.es).

“All authors, faculty, and staff in a position to control the content of this CME activity and their spouses/life partners (if any) have disclosed that they have no financial relationships with, or financial interests in, any commercial organizations pertaining to this educational activity.”

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