Livedoid Vasculopathy and Thrombotic Disorders
Summary
Livedoid vasculopathy is a rare chronic thrombotic disorder affecting the microcirculation of the dermal vessels, most often manifesting as painful reticulated erythema, recurrent ulcerations and subsequent porcelain-white atrophic scarring. Although historically classified under vasculitis, current consensus emphasises a pauci-inflammatory or non-inflammatory thrombo-occlusive process driven by local and systemic hypercoagulability. Patients typically present with livedo racemosa on the lower extremities, severe ischaemic pain and characteristic atrophie blanche. Underlying risk factors may include inherited or acquired thrombophilias, autoimmune connective-tissue diseases and malignancies. Diagnosis relies on a synthesis of clinical history, cutaneous examination and confirmatory histopathology demonstrating fibrin thrombi within dermal vessels, endothelial proliferation and hyaline degeneration. Management strategies centre on anticoagulation with agents such as low-molecular-weight heparin and direct oral anticoagulants, supplemented by immunomodulatory therapies including intravenous immunoglobulins or targeted biologic agents in refractory cases. Despite advances in understanding the molecular pathways and treatment algorithms, optimal therapeutic regimens remain to be defined, and multidisciplinary care is essential to prevent irreversible tissue damage and improve quality of life.
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Livedoid Vasculopathy and Thrombotic Disorders publication trend
The graph below shows the total number of articles in livedoid vasculopathy and thrombotic disorders across all publications each year (not limited to Nature Index journals).
Technical terms
Livedo racemosa: a persistent, netlike, reddish-blue skin pattern reflecting slowed cutaneous blood flow.
Atrophie blanche: porcelain-white, stellate scars that develop after healing of chronic skin ulcers.
Thrombophilia: a predisposition to intravascular clot formation due to inherited or acquired coagulation abnormalities.
Janus kinase inhibitor: a class of drugs that block intracellular signalling enzymes (JAK1/2) involved in inflammatory and thrombotic pathways.
Microcirculation: the network of small blood vessels, including arterioles, capillaries and venules, where exchange of gases and nutrients occurs.
References
- Livedoid vasculopathy: A review with focus on terminology and pathogenesis. Vascular Medicine (2022).
- Baricitinib is potentially effective in the treatment of refractory livedoid vasculopathy. Frontiers in Immunology (2022).
- Comparative Efficacy of Rivaroxaban and Immunoglobulin Therapy in the Treatment of Livedoid Vasculopathy: A Systematic Review. Cureus (2022).
- Adalimumab in Treating Refractory Livedoid Vasculopathy. Vaccines (2022).
- A comprehensive review on pathogenesis, associations, clinical findings, and treatment of livedoid vasculopathy. Frontiers in Medicine (2022).
About these summaries
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