Livedoid vasculopathy - Skin
The etiology of LV is likely a combination of inherited and acquired hypercoagulable states resulting in an occlusive (noninflammatory) phenomenon within the blood vessels. Examples of such inherited conditions include protein C and S deficiencies as well as factor V Leiden, prothrombin G20210A, and plasminogen activator inhibitor-1 gene mutations. Acquired conditions include anticardiolipin antibodies, lupus anticoagulant, hyperhomocysteinemia, and various disorders of platelet dysfunction.
A combination of related hypotheses propose that capillary leak from chronic venous insufficiency results in extravasated fibrinogen and the subsequent formation of a fibrin cuff around vessels leading to relative hypoxia and decreased nutrient supply to surrounding tissues. Furthermore, microthrombi and white blood cells collect within the vessel walls, releasing proteolytic substances resulting in tissue breakdown. This is complicated by the lack of tissue plasminogen activator and increases in plasminogen activator inhibitors in certain individuals.
Pediatric Patient Considerations:
The condition is rare in children.
L95.0 – Livedoid vasculitis
238762002 – Livedoid vasculitis
- Chilblains – Usually a history of cold exposure.
- Cutis marmorata telangiectatica congenita – Present since birth.
- Erythema ab igne – Look for history of chronic heat source exposure.
- Lichen sclerosus – Look for shiny atrophic plaques on anogenital regions (often in a figure-of-eight pattern) and trunk.
- Localized scleroderma (morphea) – Look for ivory indurated plaques on other areas of the body, often with a violaceous rim.
- Malignant atrophic papulosis – Look for smaller lesions on the trunk or proximal extremities, surrounded by a distinctive telangiectatic rim.
- Livedo reticularis, physiologic – Look for presence upon cold exposure and resolution with rewarming.
- Livedo reticularis, idiopathic – Diagnosis of exclusion, persistent, no known triggers, more common in young women, exaggerated upon cold exposure.
- Livedo reticularis, primary – Diagnosis of exclusion; lesions appear and disappear spontaneously without known triggers.
- Livedo reticularis, autoimmune connective tissue disorders – Look for evidence of systemic lupus erythematosus, antiphospholipid syndrome, vasculitis, dermatomyositis, etc.
- Livedo reticularis, hematologic disorders – Look for cryoglobulinemia, deep vein thrombosis, disseminated intravascular coagulation.
- Livedo reticularis, embolic or deposition – Look for dyslipidemia, septic emboli, calciphylaxis (end-stage renal failure).
- Livedo reticularis, drug-induced – Look for history of minocycline, amantadine, quinidine, etc.
- Ulcers, arterial – Look for history of arterial disease; very painful punched-out ulcers on the feet, toes, heel. Buerger disease with long, extensive smoking history is very common.
- Ulcers, venous – Look for history of extensive varicose veins, chronic venous stasis, and ulcers on the inner portion of lower leg.
- Ulcers, neuropathic – Look for history of diabetes or neuropathy.
- Ulcers, traumatic – Look for history of trauma.
- Ulcers, vasculitis – Look for leukocytoclasis on biopsy.