Livedoid vasculopathy: an in-depth analysis using a modified Delphi approach

Abstract

Livedoid vasculopathy (LV) is a noninflammatory thrombotic condition presenting in a primary idiopathic or secondary subtype associated with abnormal coagulation factors. Different from atrophie blanche (AB), which is a clinical manifestation of certain scars, LV may have AB in combination with recurrent livedo reticularis with chronic and painful skin ulcers particularly around the ankle region, and at the back of the feet. Histology is characterized by segmental hyalinizing changes at the subintimal region of small dermal vessels with thrombotic occlusions. LV skin ulcers resolve with stellate, porcelain-white scars that need to be distinguished from similar changes seen with venous insufficiency. "Atrophie blanche" was originally used synonymously with "livedoid vasculopathy." AB describes spontaneously occurring porcelain-white skin areas with red dots that typically occur in the context of skin changes attributed to chronic venous insufficiency. The 2 forms of AB--(1) the LV-AB complex and (2) AB in the context of chronic venous insufficiency--are unrelated and require separate diagnostic and therapeutic approaches. Using a modified Delphi method, we have developed an international consensus document on the diagnosis and management of LV. Individual sections of this document provide advice on diagnosis and management of LV.

Keywords: AB; IVIG; LV; PAN; atrophie; atrophie blanche; blanche; coagulopathy; intravenous immunoglobulin; livedoid; livedoid vasculopathy; lower extremity; polyarteritis nodosa; vasculopathy.