Livedoid vasculopathy: An in-depth analysis using a modified Delphi approach - 14/11/13

Doi : 10.1016/j.jaad.2013.07.019

Afsaneh Alavi, MD ^a,^⁎ , Jürg Hafner, MD ^c, Jan P. Dutz, MD ^e, Dieter Mayer, MD ^d, R. Gary Sibbald, MD ^a,^b, Paulo Ricardo Criado, MD, PhD ^f, Patricia Senet, MD ^g, Jeffery P. Callen, MD ^h, Tania J. Phillips, MD ⁱ, Marco Romanelli, MD, PhD ^j, Robert S. Kirsner, MD, PhD ^k
^a Department of Medicine (Dermatology), University of Toronto, Toronto, Ontario, Canada
^b Faculty of Public Health, University of Toronto, Toronto, Ontario, Canada
^c Dermatology, University Hospital of Zurich, Zurich, Switzerland
^d Clinic for Cardiovascular Surgery, University Hospital of Zurich, Zurich, Switzerland
^e Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, Canada
^f Dermatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Sao Paulo, Brazil
^g Assistance Publique-Hôpitaux de Paris, Dermatology Department, Hôpital Tenon, Paris, France
^h Dermatology, University of Louisville School of Medicine, Louisville, Kentucky
ⁱ Dermatology, Boston University School of Medicine, Boston, Massachusetts
^j Dermatology, University of Pisa, Pisa, Italy
^k Dermatology and Cutaneous Surgery, University of Miami, Miami, Florida

^∗Reprint requests: Afsaneh Alavi, MD, FRCPC, Dermatology and Wound Care Clinic, Women's College Hospital, 76 Grenville St, 5th Floor, Toronto, Ontario, M5S 1B2, Canada.

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.

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Key words : atrophie, blanche, coagulopathy, livedoid, lower extremity, vasculopathy

Abbreviations used : AB, IVIG, LV, PAN