Livedoid vasculopathy: A review of pathogenesis and principles of management

Indian J Dermatol Venereol Leprol. Sep-Oct 2016;82(5):478-88. doi: 10.4103/0378-6323.183635.

Abstract

Livedoid vasculopathy is a rare cutaneous disease manifesting as recurrent ulcers on the lower extremities. The ulceration results in atrophic, porcelain white scars termed as atrophie blanche. The pathogenesis is yet to be understood with the main mechanism being hypercoagulability and inflammation playing a secondary role. The important procoagulant factors include protein C and S deficiency, factor V Leiden mutation, antithrombin III deficiency, prothrombin gene mutation and hyperhomocysteinemia. Histopathology of livedoid vasculopathy is characterized by intraluminal thrombosis, proliferation of the endothelium and segmental hyalinization of dermal vessels. The treatment is multipronged with anti-thrombotic measures such as anti-platelet drugs, systemic anticoagulants and fibrinolytic therapy taking precedence over anti-inflammatory agents. Colchicine, hydroxychloroquine, vasodilators, intravenous immunoglobulin, folic acid, immunosuppressive therapy and supportive measures are also of some benefit. A multidisciplinary approach would go a long way in the management of these patients resulting in relief from pain and physical as well as psychological scarring.

Publication types

  • Review

MeSH terms

  • Disease Management*
  • Fibrinolytic Agents / therapeutic use
  • Humans
  • Lower Extremity / pathology*
  • Platelet Aggregation Inhibitors / therapeutic use
  • Skin Diseases, Vascular / diagnosis*
  • Skin Diseases, Vascular / drug therapy*

Substances

  • Fibrinolytic Agents
  • Platelet Aggregation Inhibitors