Livedoid vasculitis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]

Synonyms and keywords: Livedo reticularis; livedo vasculitis; livedoid vasculopathy; primary livedo reticularis; idiopathic livedo reticularis; vitiligo reticularis;Atrophie blanche;

Overview

Livedoid vasculitis is a vascular disorder mostly affecting women. It may be aggravated by exposure to cold and occurs most often in the lower extremities. It can also be associated with the presence of anti-cardiolipin antibodies (the Antiphospholipid syndrome).

Historical Perspective

The condition's name derives from the Latin livere meaning bluish.

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

There are no life-threatening causes of livedoid vasculitis that may result in death or permanent disability if left treated within 24 hours.

Common Causes

Causes by Organ System

Cardiovascular Antithrombin III deficiency, arteriosclerosis, Buerger's disease, carbon dioxide arteriography, cholesterol emboli syndrome, hypercalcemia, peripheral arterial occlusive disease, vasculitis
Chemical/Poisoning No underlying causes
Dental No underlying causes
Dermatologic Cutis marmorata telangiectatica congenita, dermal vascular occlusion, dermatomyositis, FILS syndrome, idiopathic livedo reticularis, polyarteritis nodosa, scleroderma, Sneddon-Champion syndrome, systemic lupus erythematosus, vasculitis
Drug Side Effect Amantadine, dextroamphetamine, gefitinib, interferon beta treatment, methylphenidate, rasagiline
Ear Nose Throat No underlying causes
Endocrine Graves hyperthyroidism, pheochromocytoma
Environmental No underlying causes
Gastroenterologic Pancreatitis
Genetic Cystathionine beta-synthase deficiency, Down syndrome, Ehlers-Danlos syndrome, familial primary cryofibrinogenemia, homocystinuria, oxalosis, plasminogen activator inhibitor-1 promoter homozygosity, protein C deficiency, prothrombin G20210A heterozygosity
Hematologic Anticardiolipin syndrome, antiphospholipid syndrome, antithrombin III deficiency, cryoglobulinemia, elevated fibrinopeptide A, factor V Leiden mutation, familial primary cryofibrinogenemia, Henoch-Schönlein purpura, increased plasminogen activator release, lupus anticoagulant, pernicious anemia, polycythaemia rubra vera, protein C deficiency, thrombocytosis
Iatrogenic Midline catheter
Infectious Disease Cytomegalovirus infection, Lyme disease, syphilis, tuberculosis
Musculoskeletal/Orthopedic Ehlers-Danlos syndrome, FILS syndrome, rheumatoid arthritis
Neurologic Anticardiolipin syndrome, antiphospholipid syndrome, mononeuritis multiplex, moyamoya disease, Sneddon-Champion syndrome
Nutritional/Metabolic Cystathionine beta-synthase deficiency, homocystinuria, hyperhomocysteinemia, primary hyperoxaluria
Obstetric/Gynecologic Anticardiolipin syndrome, antiphospholipid syndrome
Oncologic Lymphoma, metastatic breast carcinoma, pheochromocytoma, renal cell carcinoma
Ophthalmologic Cystathionine beta-synthase deficiency, homocystinuria
Overdose/Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary Churg-Strauss syndrome, sarcoidosis, scleroderma, tuberculosis
Renal/Electrolyte Henoch-Schönlein purpura, hypercalcemia, renal cell carcinoma, systemic lupus erythematosus
Rheumatology/Immunology/Allergy Anticardiolipin syndrome, antiphospholipid syndrome, Buerger's disease, Churg-Strauss syndrome, cryoglobulinemia, dermatomyositis, FILS syndrome, Henoch-Schönlein purpura, IgM antiphosphatidylserine-prothrombin complex antibody, immune complex disease, increased plasminogen activator release, lupus anticoagulant, mixed connective tissue disease, plasminogen activator inhibitor-1 promoter homozygosity, polyarteritis nodosa, polyclonal IgM hypergammopathy, rheumatoid arthritis, sarcoidosis, scleroderma, Sjögren's syndrome, systemic lupus erythematosus, vasculitis
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Miscellaneous Silicone implants

Causes in Alphabetical Order

Diagnosis

Physical Examination

Skin

Extremities

Treatment

Other than identifying and treating any underlying conditions in secondary livedo,[58] idiopathic livedo reticularis itself may improve with warming the legs, but once established the skin discolouration may become permanent.

References

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