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Livedoid vasculopathy
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Livedoid vasculopathy

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Contributors: Tyler Werbel, Patricia Ting MD, Ben Barankin MD, Susan Burgin MD
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Synopsis

Livedoid vasculopathy (LV), also known as livedoid vasculitis, is characterized by hyalinization of vessels, causing thrombotic disease with or without ulceration, which may progress to end-stage atrophic scarring known as atrophie blanche (AB) on the lower legs. LV is a rare condition, with an estimated incidence of 1:100 000 per year. The incidence increases during summer months and during pregnancy. It tends to affect middle-aged individuals and has a female-to-male ratio of 3:1.

The pathogenesis of LV is not well understood. It has been suggested that the occlusion of dermal vessels with fibrin thrombi may contribute to its development. This hypothesis stems from its association with inherited and acquired hypercoagulable states as well as various systemic illnesses. However, LV can also be idiopathic.

The condition is rare in children.

Codes

ICD10CM:
L95.0 – Livedoid vasculitis

SNOMEDCT:
238762002 – Livedoid vasculitis

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Chronic venous insufficiency with atrophie blanche
  • 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 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.

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed: 11/05/2018
Last Updated: 11/13/2018
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Livedoid vasculopathy
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Livedoid vasculopathy : Ankle, Lower leg, Atrophic scar, Feet or toes
Clinical image of Livedoid vasculopathy
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