Cutaneous Vascular Occlusion Syndromes Paul K Shitabata M

Cutaneous Vascular Occlusion Syndromes Paul K. Shitabata, M. D. Dermatopathologist 3/2/00

General Categories n n n n Microvascular platelet plugs Cold-related gelling or agglutination Local or systemic altered coagulation Vessel-invasive organisms Embolization Thrombi Miscellaneous

Microvascular Platelet Plugs n n Heparin Necrosis Paroxysmal nocturnal hemoglobinuria Thrombotic thrombocytopenic purpura Myeloproliferative disease

Heparin Necrosis n Five to ten days after start of therapy May occur immediately if previously sensitized n Underlying medical problems (HTN, CVD, DM, CA) n n n Tender, sharply demarcated plaques which become necrotic Ab to complex of heparin and PF 4 n Ig. G and Ig. M which when bound, activate platelets

Heparin Necrosis-Etiology n LMWH heparin may be less immunogenic than unfractionated heparin n n Rare cases still reported Has been observed in absence of thrombocytopenia n Overlap with heparin associated thrombocytopenia not always true

Paroxysmal Nocturnal Hemoglobinuria n n Clonal blood disorder with intravascular hemolysis, venous thrombosis, and deficient hematopoiesis Skin varies from dusky patches to ulcers Thrombi in vessels Somatic mutation in X-linked PIG-A gene (phosphatidylinositol glycan class A) n RBCs more sensitive to complement

TTP

Cold-related Gelling or Agglutination n Cryoglobulins Cryofibrinogenemia Cold agglutinin disease

Local or Systemic Coagulation Abnormalities n n n Protein C and Protein S deficiency DIC Coumadin necrosis Antiphospholipid antibody syndrome Atrophie blanche

Vessel-Invasive Organisms n n n Fungi Ecthyma gangrenosum Disseminated strongyloides

Embolization n n Cholesterol emboli Oxalate crystal deposition

Oxalate Crystal Deposition n n Two AR forms Acquired form n Increased intake or absorption, impaired renal excretion, pyridoxine deficiency, GI Crohn’s or jejunal-ileal bypass

Thrombus n n Atrial myxoma, marantic or septic endocarditis Crystalglobulin vasculopathy

Miscellaneous n n n Calciphylaxis Degos’ disease Sickle cell anemia

References n Robson KJ and Piette WW. Adv Dermatol 2000; 15: 153 -182