Vascular Disorders

James W. Patterson, M.D. University of Virginia Health System

• Antiphospholipid antibody syndrome • Sneddon’s syndrome • Cryoglobulinemia • Factor V Leiden mutation Coagulopathies

• Protein C and S deficiency – Disseminated intravascular coagulation – Coumarin necrosis – Purpura fulminans • Thrombotic thrombocytopenic purpura • Heparin necrosis occurs through development of anti-platelet antibodies Antiphospholipid Antibody

Monoclonal Cryoglobulinemia DIC

Heparin Necrosis Livedo Reticularis

• Net-like pattern of red-blue mottling of skin, with white areas inbetween • Can present with Sneddon’s syndrome • Idiopathic • erythematosus • Cutaneous polyarteritis nodosa • • Atrophie blanche Livedo Reticularis - Pathology

• Microscopic findings vary, depending on cause… – PAN: – Sneddon’s syndrome: arterial thrombi • Controversy about where to (white areas or areas of mottled erythema) Atrophie Blanche

• Fibrin thrombi and hyalinization of vessels • Ulcers heal to form stellate scars • Decreased fibrinolytic activity • Anti-cardiolipin antibodies, lupus anticoagulant, protein C deficiency, factor V Leiden mutation

Embolic Disease

• Livedo reticularis or purpura with necrosis, ulceration, or gangrene • Cholesterol emboli • Tumor emboli • Septic emboli

Degos’ Disease

• Discrete red papules with “porcelain” white centers • Death from peritonitis, cerebral infarcts • May be fulminant or chronic • Possible causes: vascular occlusion from endothelial defect, hypercoagulable state, defects in platelet aggregation or fibrinolytic activity, anticardiolipin antibodies

Leukocytoclastic Vasculitis

• Endothelial swelling • Permeation by inflammatory cells • Extravasated erythrocytes • Leukocytoclasis • Fibrin deposition • Causes: infection, drugs, connective tissue diseases, malignancy, mixed cryoglobulinemia

Polyarteritis Nodosa

• Involves small and medium-sized • Disease associations: hepatitis B and C, streptococcal infections, inflammatory bowel disease, LE • Cutaneous variant presents as livedo reticularis, may ulcerate, tends t5o be long- term, relapsing course

Microscopic Polyangiitis

• Vasculitis involving • Also involves and post- venules • Is an ANCA-positive disease (most often, p- ANCA) • Pulmonary involvement but not

• Forms include Buerger’s disease, Mondor’s disease • Superficial Migratory Thrombophlebitis – Cords on lower legs or elsewhere – Association with malignancy (Trousseau’s syndrome), especially pancreeas, , stomach, prostate, hematopoietic • May be promoted by hypercoagulable state

PAN versus Thrombophlebitis

PAN

Thrombophlebitis Eosinophilic Vasculitis

• Probably a phenomenon or variant form of vasculitis rathr than a specific entity • Associated with hypereosinophilic syndrome, rheumatoid arthritis, other connective tissue diseases

Lymphocytic Vasculitis

• Controversial term; should be limited to a few conditions: – Associated with lupus erythematosus – Pityriasis lichenoides – Pigmented purpuric eruptions – Perniosis – Infectious exanthems (Rocky Mountain spotted fever) Pityriasis Lichenoides Pigmented Purpura Perniosis Granulomatosis with Polyangiitis

• Formerly Wegener’s granulomatosis • Necrotizing granulomatous disease involving upper and lower respiratory tract and kidneys • Skin involvement in 40-50% of cases, with nodules, ulcers, petechial or purpuric eruptions • Most patients positive for c-ANCA

Eosinophilic Granulomatosis with Polyangiitis • Also known as Churg-Strauss syndrome • Asthma, allergic rhinitis, fever, eosinophilia, pulmonary infiltration • Cutaneous include leukocytoclastic vasculitis, nodules, urticaria, livedo • Positive ANCA, usually p- but occasionally c- • Triggers include drugs • Recent reports of Churg-Strauss-like syndrome in patients receiving leukotriene receptor antagonists

Granuloma Faciale

• Nodules on face, usually solitary, reddish brown in color • Considered a small vessel vasculitis • Treatments include topical steroids, cryotherapy, laser

Erythema Elevatum Diutinum

• Brownish-red nodules over extensors; progressively harden over time • Association with keratitis • Reported with HIV infection • May be related to “papular neutrophilic xanthoma” of the face

Papular Neutrophilic Xanthoma Sweet’s Syndrome

• Tender, sharply marginated erythematous plaques or nodules • Fever and leukocytosis • Associations: URI, hematologic malignancy, inflammatory bowel disease treated with GCSF • Similarities to pyoderma gangrenosum

Rheumatoid Neutrophilic Dermatosis • Accompanies well established rheumatoid arthritis • Urticarial plaques on trunk or extremities • Some cases show changes of interstitial granulomatous dermatitis

Pyoderma Gangrenosum

• Pustule or nodule breaks down and ulcerates; spreads when traumatized • Bullous variant exists • Another variant termed “superficial granulomatous pyoderma” • Causes: idiopathic, inflammatory bowel disease, rheumatoid arthritis, leukemia, monoclonal gammopathy

Behçet’s Syndrome

• Oral-genital ulcers, uveitis, arthritis, meningoencephalitis • Skin findings include papulo-pustules, erythema nodosum, pathergic lesions • Strong association with HLA types Bw51 and B12

Pathergy