Warfarin Therapy for Livedoid Vasculopathy Associated With Cryofibrinogenemia and Hyperhomocysteinemia

Browning, Catherine E.; Callen, Jeffrey P.

doi:10.1001/archderm.142.1.75

Cited by 55 publications

(60 citation statements)

References 28 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Successful treatment of LV with thrombolytic therapy such as heparin sodium, warfarin sodium (Coumadin), and tissue plasminogen activator (tPA) supports this hypothesis. [6][7][8][9] Plasminogen activator inhibitor-1 (PAI-1) is an important inhibitor of the fibrinolytic system. Composed of a singlechain glycoprotein, it is the primary inhibitor of both tPA and urokinase-type plasminogen activator.…”

Section: Discussionmentioning

confidence: 99%

Livedoid Vasculopathy Associated With Plasminogen Activator Inhibitor-1 Promoter Homozygosity (4G/4G) Treated Successfully With Tissue Plasminogen Activator

et al. 2006

View full text Add to dashboard Cite

Background: Livedoid vasculopathy (LV) is an occlusive thrombotic disease that affects primarily the small blood vessels of the lower extremities and often is associated with recurrent painful ulcerations. The pathogenesis of LV is unclear, but the disease is largely attributed to a hypercoagulable state. Factor V Leiden mutation, heterozygous protein C deficiency, homozygous hyperhomocysteinemia, and other inherited thrombophilias have been associated with LV. Plasminogen activator inhibitor-1 (PAI-1) is an important inhibitor of the fibrinolytic system. Elevated levels of PAI-1 are found in some patients with thrombotic diseases. Some of these patients are homozygous for an allele of PAI-1 containing a stretch of 4 guanines at base −675 in the promoter region. This variant is associated with elevated PAI-1 protein levels, impaired fibrinolysis, and increased risk of thrombosis.Observations: A 33-year-old white woman had a 3-month history of painful enlarging ulcers on both ankles. Various therapies, including administration of oral antibiotic agents and prednisone up to 100 mg/d, to treat presumed vasculitis, were unsuccessful. Skin biopsy specimens revealed numerous thick-walled small blood ves-

show abstract

Section: Discussionmentioning

confidence: 99%

Livedoid Vasculopathy Associated With Plasminogen Activator Inhibitor-1 Promoter Homozygosity (4G/4G) Treated Successfully With Tissue Plasminogen Activator

et al. 2006

View full text Add to dashboard Cite

show abstract

“…3 Treatment for primary CF with reported success includes use of fibrinolytics (eg, streptokinase), 3,[16][17][18][19][20] immunomodulators (eg, glucocorticoids in combination with azathioprine or chlorambucil, and plasmapheresis), 5,[21][22][23][24] and anticoagulants (eg, such as heparin and warfarin). 14,25 Aspirin and colchicine have been shown to be ineffective. 5 Treatment for secondary CF is directed by screening and evaluation for possible underlying infection, malignancy or inflammatory disease, and specific treatment of the underlying disease.…”

Section: Discussionmentioning

confidence: 99%

Untitled

Chen

Sreenivasan²,

Shojania³

et al. 2015

Exp Clin Transplant

View full text Add to dashboard Cite

Cryofibrinogenemia is a rare disorder in which plasma, not serum, forms a cryoprecipitate. Patients with cryofibrinogenemia may be asymptomatic, or they may have painful ulcers, purpura, livedo reticularis, Raynaud phenomenon, perniosis of the extremities, thrombosis, and arthralgia. Cryofibrinogenemia may be primary or secondary to an underlying disorder such as connective tissue disease, malignancy, infection, drugs, or thromboembolic disease.Here, we present a 41-year-old woman with a pancreatic neuroendocrine tumor who underwent a Whipple procedure in 2003 followed by 2 liver transplants for hepatic metastases. Three years posttransplant, we discovered a biopsy-proven metastatic lesion in her femur. Five years posttransplant, she developed acute, severe pain in both feet, and was found to have cryofibrinogenemia despite immunosuppression posttransplant. Testing for connective tissue diseases and hematologic malignancy were negative. She was treated with high-dose prednisone, which completely resolved her symptoms. We also conducted a review of the literature via a PubMed search to summarize the association of cryofibrinogenemia with malignancy and treating cryofibrinogenemia with corticosteroids.Our study is the first reported case of cryofibrinogenemia that developed secondary to a neuroendocrine tumor posttransplant. Our report suggests that cryofibrinogenemia may occur despite immunosuppression adequate to prevent graft rejection, and that high-dose corticosteroids are an effective treatment for posttransplant cryofibrinogenemia.

show abstract

“…9,[12][13][14][15][16] Changes in the blood flow not only include venous insufficiency, but also diseases that lead to hyperviscosity syndrome such as chronic myelogenous leukemia, cryoglobulinemia and heavy chain disease. 9,17 Hypercoagulable states are rare situations and still partially unknown.…”

Section: Etiopathogenesismentioning

confidence: 99%

“…8,42 Vitamin K antagonist oral anticoagulants, especially warfarin, have been used by several authors. 8,12,19,50,51,53,56,69 The INR (International Normalized Ratio) is usually maintained between 1.5 and 2.0. 8,12 However, it is sometimes difficult to manage the use of oral anticoagulants, given the frequent drug interactions and the patient's diet interference (supply of vitamin K in food).…”

Section: (Vi) Therapies Of Still Poorly Understood Mechanismsmentioning

confidence: 99%

“…8,12,19,50,51,53,56,69 The INR (International Normalized Ratio) is usually maintained between 1.5 and 2.0. 8,12 However, it is sometimes difficult to manage the use of oral anticoagulants, given the frequent drug interactions and the patient's diet interference (supply of vitamin K in food). This makes it hard to maintain the INR levels and leads us to reserve the use of these agents for special situations, such as for individuals with LV and recurrent episodes of thrombosis, due to genetic mutations related to thrombophilia or antiphospholipid syndrome.…”

Section: (Vi) Therapies Of Still Poorly Understood Mechanismsmentioning

confidence: 99%

See 1 more Smart Citation

Vasculopatia livedoide: uma doença cutânea intrigante

Criado

Rivitti

Sotto

et al. 2011

An. Bras. Dermatol.

View full text Add to dashboard Cite

Livedoid vasculopathy is a skin disease that occludes the blood vessels of the dermis. It has a pauciinflammatory or non-inflammatory nature. It is characterized by the presence of macular or papular, erythematous-purpuric lesions affecting the legs, especially the ankles and feet, and producing intensely painful ulcerations, which cause white atrophic scars called "atrophie blanche". This review includes studies and case reports found in the medical literature regarding the etiopathogenic associations of the disease, particularly those related to thrombophilia, their histopathological findings and the therapeutic approaches used in the difficult clinical management of these cases. Keywords: Leg ulcer; Livedo reticularis; Thrombophilia; Thrombosis; Venous thrombosis Resumo: A vasculopatia livedoide é uma afecção cutânea oclusiva dos vasos sanguíneos da derme, de caráter pauci-inflamatório ou não-inflamatório. Caracteriza-se pela presença de lesões maculosas ou papulosas, eritêmato-purpúricas, nas pernas, especialmente nos tornozelos e pés, as quais produzem ulcerações intensamente dolorosas, que originam cicatrizes atróficas esbranquiçadas, denominadas "atrofia branca". Nesta revisão, abordamos os estudos e relatos de caso da literatura médica referentes às associações etiopatogênicas da doença, particularmente as que se referem aos estados de trombofilia, seus achados histopatológicos e abordagens terapêuticas empregadas na difícil condução clínica destes casos.

show abstract

Warfarin Therapy for Livedoid Vasculopathy Associated With Cryofibrinogenemia and Hyperhomocysteinemia

Cited by 55 publications

References 28 publications

Livedoid Vasculopathy Associated With Plasminogen Activator Inhibitor-1 Promoter Homozygosity (4G/4G) Treated Successfully With Tissue Plasminogen Activator

Livedoid Vasculopathy Associated With Plasminogen Activator Inhibitor-1 Promoter Homozygosity (4G/4G) Treated Successfully With Tissue Plasminogen Activator

Untitled

Vasculopatia livedoide: uma doença cutânea intrigante

Contact Info

Product

Resources

About