warfarin and Cryoglobulinemia

Palpable purpura tends to indicate involvement of small vessel vasculitis in the upper dermis. Livedo racemosa, nodular lesion and skin ulceration are indicative of involvement of small to medium-sized vessel vasculitis in the lower dermis to subcutaneous fat. We set out to establish a new algorithm (KAWAKAMI algorithm) for primary cutaneous vasculitis based on the Chapel Hill Consensus Conference classification and our research results, and apply to the diagnosis. The first step is to measure serum antineutrophil cytoplasmic antibodies (ANCA) levels. If myeloperoxidase-ANCA is positive, Churg-Strauss syndrome or microscopic polyangiitis can be suspected, and if the patient is positive for proteinase 3-ANCA, Wegener's granulomatosis is most likely. Next, if cryoglobulin is positive, cryoglobulinemic vasculitis should be suspected. Third, if direct immunofluorescence of the skin biopsy specimen reveals immunoglobulin A deposition within the affected vessels, Henoch-Schönlein purpura is indicated. Finally, the presence of anti-phosphatidylserine-prothrombin complex antibodies and/or lupus anticoagulant and histopathological necrotizing vasculitis in the upper to middle dermis (leukocytoclastic vasculitis) indicates cutaneous leukocytoclastic angiitis, whereas if necrotizing vasculitis exists in the lower dermis and/or is associated with the subcutaneous fat, cutaneous polyarteritis nodosa is indicated. The KAWAKAMI algorithm may allow us to refine our earlier diagnostic strategies and allow for efficacious treatment of primary cutaneous vasculitis. In cutaneous polyarteritis nodosa, warfarin or clopidogrel therapies should be administrated, and in cases that have associated active inflammatory lesions, corticosteroids or mizoribine (mycophenolate mofetil) therapy should be added. We further propose prophylactic treatment of renal complications in patients with Henoch-Schönlein purpura.

Topics: Adrenal Cortex Hormones; Algorithms; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antibodies, Antineutrophil Cytoplasmic; Clopidogrel; Cryoglobulinemia; Diagnosis, Differential; Humans; IgA Vasculitis; Immunoglobulin A; Myeloblastin; Peroxidase; Polyarteritis Nodosa; Ribonucleosides; Ticlopidine; Vasculitis, Leukocytoclastic, Cutaneous; Warfarin

Topics: Adult; Anabolic Agents; Anti-Inflammatory Agents; Anticoagulants; Cryoglobulinemia; Cyclophosphamide; Female; Humans; Immunosuppressive Agents; Prednisone; Stanozolol; Warfarin

Topics: Adrenal Cortex Hormones; Aged; Anticoagulants; Cryoglobulinemia; Drug Therapy, Combination; Humans; Leg Ulcer; Male; Treatment Outcome; Warfarin

Topics: Antibodies, Anticardiolipin; Anticoagulants; Cryoglobulinemia; Debridement; Enoxaparin; Hepatitis C; Humans; Male; Middle Aged; Necrosis; Penis; Plastic Surgery Procedures; Vasculitis, Leukocytoclastic, Cutaneous; Venous Thrombosis; Warfarin

Livedoid vasculopathy is an idiopathic, chronic disorder manifested by painful, purpuric macules on the lower extremities that superficially ulcerate, resulting in atrophic, stellate scars with peripheral telangiectasias and hyperpigmentation.. A 50-year-old man presented with recurrent, painful ulcerations on the medial aspect of his malleoli and calves. The clinical presentation, histologic findings, and results of laboratory evaluation confirmed the diagnosis of livedoid vasculopathy in this case. Despite being refractory to treatment with multiple other medications, the lesions responded dramatically to oral warfarin sodium therapy.. Treatment with warfarin may be a beneficial therapy for patients with livedoid vasculopathy.

Topics: Anticoagulants; Biopsy; Cryoglobulinemia; Cryoglobulins; Diagnosis, Differential; Fibrinogens, Abnormal; Follow-Up Studies; Humans; Hyperhomocysteinemia; Male; Middle Aged; Skin; Skin Diseases, Vascular; Warfarin