Livedoid vasculopathy

INTRODUCTION

Livedoid vasculopathy is a chronic, recurrent, painful skin disorder that involves the distal lower extremities and feet. The disorder is a thrombotic, noninflammatory condition associated with the formation of clots within the lumina of medium-sized arterioles and with hyalinizing changes within the subintimal region of involved vessels.

Livedoid vasculopathy can occur by itself or in the setting of a variety of systemic disorders, including systemic lupus erythematosus (SLE) and the antiphospholipid syndrome (APS). The disorder has also been linked to a growing list of clotting abnormalities. Livedoid vasculopathy is often misdiagnosed as either chronic venous insufficiency or some form of systemic or cutaneous vasculitis.

The clinical manifestations, diagnosis, and treatment of livedoid vasculopathy are presented here. The diagnosis and treatment of chronic venous insufficiency, as well as the diagnosis and treatment of small- and medium-vessel vasculitis of the skin, are discussed elsewhere. (See "Diagnostic evaluation of chronic venous insufficiency" and "Medical management of lower extremity chronic venous disease" and "Hypersensitivity vasculitis in adults" and "Clinical manifestations and diagnosis of polyarteritis nodosa in adults".)

NOMENCLATURE

The literature related to livedoid vasculopathy is highly problematic with regard to nomenclature [1]. There are three major sources of confusion:

  • Atrophie blanche, the descriptive term for the major cutaneous lesions associated with this disorder, is often employed interchangeably with the more appropriate names for the disease entity, livedoid vasculopathy or segmental hyalinizing vasculopathy [2]. (See 'Atrophie blanche' below.)
  • The terms “livedoid vasculitis” or just “livedo vasculitis” have been used widely to describe this condition, but, in fact, the disorder does not involve destructive inflammation within the blood vessel wall and is, therefore, not a true vasculitis.
  • A host of names for livedoid vasculopathy have been employed in the literature:

                     

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Literature review current through: Nov 2014. | This topic last updated: May 20, 2013.
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References
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