Thrombotic skin disease as a marker of the anticardiolipin syndrome: Livedo vasculitis and distal gangrene associated with abnormal serum antiphospholipid activity

doi:10.1016/S0190-9622(89)70133-X

Journal of the American Academy of Dermatology

Volume 20, Issue 6, June 1989, Pages 1063-1069

https://doi.org/10.1016/S0190-9622(89)70133-X Get rights and content

Two patients with livedo vasculitis and microthrombosis of the dermal vessels and one patient with symmetric gangrene of both hands are presented. Their only biologic abnormality was the presence of a lupus-type circulating anticoagulant and an increased level of anticardiohpin antibodies. These observations and the few previously reported cases of cutaneous disorders associated with abnormal antiphospholipid activity suggest that the common pathologic process is thrombosis.

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Cited by (58)

Livedoid vasculopathy
2014, Piel
The skin and hypercoagulable states
2013, Journal of the American Academy of Dermatology
Citation Excerpt :
There are many proposed mechanisms of pathogenesis of LV, including venous flow abnormalities, vascular endothelial dysfunction, immune complex deposition, and microthrombi of the dermal vessels secondary to HS.79 The reported associated HS include FV Leiden mutation,80-82 prothrombin gene mutation,83,84 antithrombin III deficiency,85 protein C deficiency,86,87 double heterozygous FV Leiden and prothrombin mutations,88 APLAs,89,90 APLS,91 and hyperhomocysteinemia.92-94 Chronic venous ulceration (CVU) often occurs in the setting of chronic venous insufficiency, varicose veins, or both.
Hypercoagulable states (HS) are inherited or acquired conditions that predispose an individual to venous and/or arterial thrombosis. The dermatologist can play a vital role in diagnosing a patient's HS by recognizing the associated cutaneous manifestations, such as purpura, purpura fulminans, livedo reticularis, livedo vasculopathy (atrophie blanche), anetoderma, chronic venous ulcers, and superficial venous thrombosis. The cutaneous manifestations of HS are generally nonspecific, but identification of an abnormal finding can warrant a further workup for an underlying thrombophilic disorder. This review will focus on the basic science of hemostasis, the evaluation of HS, the skin manifestations associated with hypercoagulability, and the use of antiplatelet and anticoagulant therapy in dermatology.
The vasculopathic reaction pattern
2009, Weedon's Skin Pathology: Third Edition
Dermatologic aspects of Antiphospholipid Antibody Syndrome
2006, Handbook of Systemic Autoimmune Diseases
Citation Excerpt :
The purpura is frequently necrotic and it may precede the livedoid vasculitis-like ulcers by several years. ( Grob and Bonerandi, 1989; Francès et al., 2005). Lesions similar to those of malignant atrophic papulosis may also be included in pseudovasculitis manifestations.
This chapter describes dermatologic aspects of antiphospholipid antibody syndrome (APS). The first definition of the APS described recurrent arterial and venous thrombosis and miscarriages in association with positive tests for the lupus anticoagulant, antibodies to negatively charged phospholipids (mainly cardiolipin) and to one of the body's natural anticoagulants, a glycoprotein (B2GP1). These remain the leading clinical features of this syndrome although its spectrum has broadened and has been expanded considerably over the last 15 years. The dermatological manifestations of the APS may be the presenting feature of the APS syndrome. They are extremely diverse and heterogeneous, ranging from minor signs to life-threatening conditions such as widespread cutaneous necrosis. Livedo reticularis (LR) is strongly associated with a subset of the APS. More scientific data are required to determine the optimum management of these patients, who might certainly benefit from recently developed newer antithrombotic agents.
Livedoid vasculopathy with combined thrombophilia: Efficacy of iloprost
2002, Revue de Medecine Interne
Introduction. – La vasculopathie livédoïde est une affection cutanée douloureuse des extrémités inférieures associant des papules purpuriques, un livedo, des ulcères chroniques et une atrophie blanche secondaire des téguments. Elle doit être distinguée des vascularites cutanées.
Exégèse. – Nous rapportons l’observation d’un homme âgé de 38 ans admis pour douleurs insomniantes des pieds associées à un livedo et à des lésions purpuriques pulpaires et des bords externes. La biopsie cutanée identifiait des thromboses multiples des vaisseaux dermiques avec prolifération endothéliale. Les tests biologiques révélaient la présence d’un anticoagulant lupique ainsi qu’une mutation hétérozygote (G1691A) du gène du facteur V (Leiden). L’anticoagulation du patient n’a pas eu d’effet antalgique. L’introduction de l’iloprost, un analogue de la prostacycline (Ilomédine®), par voie intraveineuse permettait une sédation rapide des douleurs et une disparition des lésions cutanées.
Conclusion. – La vasculopathie livédoïde est une affection cutanée liée à l’existence de processus vasculaires thrombotiques où la thrombophilie joue un rôle essentiel. L’iloprost semble être une alternative thérapeutique intéressante pour la douleur en cas d’échec des traitements anticoagulants.
Introduction. – Livedoid vasculopathy is characterized by early, focal painful purpuric lesions of the lower skin extremities without histologic finding of small vessel vasculitis.
Exegesis. – A 38-year-old man was seen in our unit for painful purpuric lesions of both feet localized on toes and external sides. Skin biopsy showed dermic vessel thrombosis and endothelial cell proliferation. Lupus anticogulant antibody was positive in association with a heterozygous factor V (Leiden) gene mutation (G1691A). Anticoagulation failed to relieve pain and cutaneous lesions. Intravenous iloprost, a prostacylcin analogous (Ilomedine®) was dramatically and rapidly effective in our patient.
Conclusion. – Livedoid vasculopathy is a cutaneous affection related to vascular thrombotic events in which thrombophilia plays a central role. Iloprost might be an interesting alternative treatment of painful purpuric lesions when anticoagulant treatments are ineffective.
Livedo (livedoid) vasculitis and the factor V Leiden mutation: Additional evidence for abnormal coagulation
2002, Journal of the American Academy of Dermatology

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¹: From the Hôpital Ste. Marguerite.

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Thrombotic skin disease as a marker of the anticardiolipin syndrome: Livedo vasculitis and distal gangrene associated with abnormal serum antiphospholipid activity

Lancet

J Am Acad Dermatol

Thrombosis in patients with the lupus antioagulant

Ann Intern Med

Lupus anticoagulant in pregnancy

Br J Obstet Gynaecol

Antibody to cardiolipin is a predictor of fetal distress or death in pregnant patients with lupus erythematosus

N Engl J Med

Lupus anticoagulant, thrombosis, and fetal loss

N Engl J Med

The anticardiolipin syndrome

J Rheumatol

Livedo reticularis associated with increased titers of anticardiolipin antibodies in systemic lupus erythematosus

Arch Dermatol

Gangrene developing after minor surgery in a patient with undiagnosed systemic lupus and lupus anticoagulant

Ann Rheum Dis

Widespread cutaneous necrosis associated with the lupus anticoagulant

Clin Exp Dermatol

Degos disease. Association with anticardiolipin antibodies and the lupus anticoagulant

Br Med J

Measurement of anticardiolipin antibodies by an enzyme-linked immunosorbent assay (ELISA): standardization and quantification of results

Clin Exp Immunol