Livedoid vasculopathy (LV), also known as livedoid vasculitis, presents with retiform purpura or ulcers on the lower legs that may progress to atrophie blanche (AB). LV is caused by vaso-occlusion of the cutaneous microcirculation that manifests histopathologically as hyalinization of vessels leading to fibrin thrombi. LV is a rare condition, with an estimated incidence of 1:100 000 per year and an increased incidence during summer months and during pregnancy. It tends to affect young to middle-aged adults, with a median age of onset of 30-50 years, and has a female-to-male ratio of 2-3:1.
The pathogenesis of LV is not well understood, but it is likely related to increased coagulability and/or decreased fibrinolysis. The strongest risk factor is a hypercoagulable state with laboratory evaluation positive for a known cause of thrombophilia in 40%-50% of patients in retrospective cohort studies. The second most common risk factor is connective tissue disease. LV can also be associated with monocolonal gammopathy, with hepatitis B or C, or can be idiopathic.
LV follows a chronic time course, with lesions often developing for months before a definitive diagnosis is made. In a recent case series, the median interval between symptom onset and diagnosis was 10 months and between onset and treatment was 22.5 months.
The condition is rare in children.
Livedoid vasculopathy
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Synopsis
Codes
ICD10CM:
L95.0 – Livedoid vasculitis
SNOMEDCT:
238762002 – Livedoid vasculitis
L95.0 – Livedoid vasculitis
SNOMEDCT:
238762002 – Livedoid vasculitis
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Differential Diagnosis & Pitfalls
- Chronic venous insufficiency with AB
- Chilblains – Usually a history of cold exposure.
- Erythema ab igne – Look for history of chronic heat source exposure.
- Lichen sclerosus – Look for shiny, atrophic plaques on the 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 – This is a diagnosis of exclusion. The condition is persistent, with no known triggers. It is more common in young women and exaggerated upon cold exposure.
- Livedo reticularis, primary – This is a 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 occur on the feet, toes, or heels. Buerger disease with a 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 the lower leg.
- Ulcers, neuropathic – Look for history of diabetes or neuropathy.
- Ulcers, traumatic – Look for history of trauma.
- Ulcers, vasculitis – Leukocytoclastic vasculitis and polyarteritis nodosa may have ulcerations as part of their presentation.
- Calciphylaxis – Commonly on fat-bearing areas of the body but can present on the lower extremities. Characterized by exquisitely painful retiform purpura.
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Last Reviewed:06/03/2020
Last Updated:06/04/2020
Last Updated:06/04/2020
Livedoid vasculopathy