ReviewThe skin and hypercoagulable states
Section snippets
Hemostasis
Hemostasis is the physiologic response that minimizes blood loss while maintaining blood flow after an injury to a vessel. Endothelial injury triggers immediate vasoconstriction and exposes tissue factor (TF) and collagen in the subendothelial matrix, providing a surface for platelet adherence and plug formation. Traditionally, coagulation has been described as a cascade of enzyme reactions, consisting of the extrinsic and intrinsic pathways converging on the common pathway to produce an
Hypercoagulable states
Venous and arterial thromboses are major causes of morbidity and mortality. Venous thromboembolism (VTE) includes deep venous thrombosis (DVT) and pulmonary embolism (PE). Hypercoagulability, in addition to stasis (slow flow) and endothelial injury, compose the Virchow triad of risk factors for venous thrombosis.6 Several components can contribute to a thrombophilic state, including genetic and acquired risk factors (Table I), triggering factors, and/or a lack of appropriate pharmacologic or
Purpura
Purpura consists of 5- to 20-mm nonblanching erythematous to violaceous macules, often located on the lower extremities. Many HS produce noninflammatory purpura with microvascular occlusion (Fig 2, A) and the associated conditions include: PF, thrombotic thrombocytopenic purpura (TTP), warfarin (Coumadin) necrosis, heparin-induced thrombotic thrombocytopenia, calciphylaxis, and catastrophic APLA syndrome (APLS).
Purpura fulminans
PF (Fig 2) is a dermatologic emergency and presents as multiple violaceous,
The use of antiplatelet and anticoagulant therapy in dermatology
The pharmacologic treatments available for VTE include antiplatelet and anticoagulant medications (Table IV) and thrombolytic agents. Embolectomy may be performed to remove large thromboses and inferior vena cava filters can be placed to help prevent additional pulmonary emboli.
In dermatology, the most frequently reported use of antiplatelet and anticoagulant medication is for the treatment of LV. There have been reports of the use of unfractionated heparin or LMWH, such as enoxaparin92, 108,
Conclusion
Primary and secondary HS are associated with cutaneous manifestations, including purpura, PF, LR, LV (atrophie blanche), anetoderma, SVT, and chronic venous ulcers. The dermatologist can identify these abnormal findings and pursue a workup for an underlying HS. The successful use of numerous antiplatelet and anticoagulant medications have been reported in patients with HS and skin manifestations, however, prospective studies are still needed to determine the optimal treatment for many of these
References (118)
A cell-based model of coagulation and the role of factor VIIa
Blood Rev
(2003)Advances in understanding pathogenic mechanisms of thrombophilic disorders
Blood
(2008)- et al.
A common genetic variation in the 3'-untranslated region of the prothrombin gene is associated with elevated plasma prothrombin levels and an increase in venous thrombosis
Blood
(1996) - et al.
Homocysteine, MTHFR and risk of venous thrombosis: a meta-analysis
J Thromb Haemost
(2005) - et al.
Diagnosis and management of neonatal purpura fulminans
Semin Fetal Neonatal Med
(2011) - et al.
Factor V Leiden and G20210A prothrombin mutation and the risk of subclavian vein thrombosis in patients with breast cancer and a central venous catheter
Ann Oncol
(2004) - et al.
Venous thromboembolism associated with pregnancy and hormonal therapy
Best Pract Res Clin Haematol
(2012) - et al.
Thromboembolic disorders in obstetrics
Best Pract Res Clin Obstet Gynaecol
(2012) Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura
Blood
(2008)- et al.
Warfarin-induced skin necrosis
J Am Acad Dermatol
(2009)
Heparin-induced thrombocytopenia: a stoichiometry-based model to explain the differing immunogenicities of unfractionated heparin, low-molecular-weight-heparin, and fondaparinux in different clinical settings
Thromb Res
Development of antibodies specific to polyanion-modified platelet factor 4 during treatment with fondaparinux
J Thromb Haemost
Calciphylaxis is usually non-ulcerating: risk factors, outcome and therapy
Kidney Int
Possible role of hypercoagulability in calciphylaxis: review of the literature
J Am Acad Dermatol
Calciphylaxis and antiphospholipid antibody syndrome
J Am Acad Dermatol
Cutaneous necrosis from calcific uremic arteriolopathy
Am J Kidney Dis
International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS)
J Thromb Haemost
Primary anetoderma associated with the antiphospholipid syndrome
J Am Acad Dermatol
Anetoderma and its prothrombotic abnormalities
J Am Acad Dermatol
Anetoderma associated with primary antiphospholipid syndrome
J Am Acad Dermatol
Thrombosis-induced ulcerations of the lower legs with coexistent anetoderma due to anti-thrombin III deficiency
J Am Acad Dermatol
Livedo reticularis: an update
J Am Acad Dermatol
Livedoid vasculopathy as a coagulation disorder
Autoimmun Rev
Livedo (livedoid) vasculitis and the factor V Leiden mutation: additional evidence for abnormal coagulation
J Am Acad Dermatol
Thrombotic skin disease as a marker of the anticardiolipin syndrome: livedo vasculitis and distal gangrene associated with abnormal serum antiphospholipid activity
J Am Acad Dermatol
Homocysteinemia and livedoid vasculitis
J Am Acad Dermatol
The prevalence of thrombophilia in patients with chronic venous leg ulceration
J Vasc Surg
Anticoagulant treatment for superficial venous thrombosis
Dis Mon
Waterfall sequence for intrinsic blood clotting
Science
An enzyme cascade in the blood clotting mechanism, and its function as a biochemical amplifier
Nature
A cell-based model of hemostasis
Thromb Haemost
Remodeling the blood coagulation cascade
J Thromb Thrombolysis
Venous and arterial thrombosis–pathogenesis and the rationale for anticoagulation
Thromb Haemost
Risk of recurrent venous thromboembolism in patients with common thrombophilia: a systematic review
Arch Intern Med
Mutation in blood coagulation factor V associated with resistance to activated protein C
Nature
The Leiden thrombophilia study (LETS)
Thromb Haemost
Hereditary coagulopathies: practical diagnosis and management for the plastic surgeon
Plast Reconstr Surg
The 20210 G→A mutation in the 3'-untranslated region of the prothrombin gene and the risk for arterial thrombotic disease
Br J Haematol
Prevalence of antithrombin deficiency in the healthy population
Br J Haematol
Risk factors for deep vein thrombosis and pulmonary embolism: a population-based case-control study
Arch Intern Med
A prospective study of venous thromboembolism after major trauma
N Engl J Med
Obesity, hemostasis and the fibrinolytic system
Obes Rev
American Heart Association call to action: obesity as a major risk factor for coronary artery disease
Circulation
Obesity and hemostasis
Arch Gynecol Obstet
Effect of leptin in arterial thrombosis following vascular injury in mice
JAMA
Mechanisms of thrombus formation
N Engl J Med
Breast cancer as an acquired thrombophilic state
J Breast Cancer
Thrombophilia in cancer
Semin Thromb Hemost
New insights into cancer-associated thrombosis
Arterioscler Thromb Vasc Biol
Thrombotic complications of central venous catheters in cancer patients
Oncologist
Cited by (0)
Funding sources: None.
Conflicts of interest: None declared.