Livedoid vasculopathy

Livedoid vasculopathy is also known as ‘livedo vasculitis’, ‘livedoid vasculitis’ and ‘livedo reticularis with summer/winter ulceration’.

What is livedoid vasculopathy?

Livedoid vasculopathy is a rare, chronic vascular disorder characterised by persistent painful ulceration of the lower extremities. The condition occurs chiefly but not exclusively on the lower leg or foot and occurs most commonly in middle-aged women. There is often an increased incidence during the winter and summer months.

Livedoid vasculopathy affects the lower legs, ankles, and/or upper surfaces of the feet. Characteristics include:

• Painful red or purple marks and spots that progress to small, tender, irregular ulcers (30% of cases)
• Painless atrophie blanche scars

Livedoid vasculopathy

What is the cause of livedoid vasculopathy?

The cause of livedoid vasculopathy remains unclear. Platelet, coagulation, and fibrinolytic disorders have been reported in some cases of livedo vasculitis, including lupus anticoagulant, protein C deficiency and factor V mutation (Leiden). These may result in blood clots in the small vessels of the lower legs.

Internal forms of vasculitis or other systemic disease are uncommon, but patients may have associated livedo reticularis with venous insufficiency causing recurrent leg ulcers, arteriosclerosis, antiphospholipid syndrome or systemic lupus erythematosus.

The course is chronic with spontaneous remissions and exacerbations. Reports of disease duration have ranged from 2.5 months to 21 years.

What is the treatment?

The main goal of therapy in livedoid vasculopathy is to reduce pain, ulceration and the development of atrophie blanche.

• Protect the area from all knocks, abrasions, known allergens/irritants and ill-fitting shoes to avoid ulceration
• Remove slough and dead tissue from the ulcers
• Treat infection (cellulitis) with antibiotics
• Leg elevation, compression therapy, bed rest, and occlusive wound dressings may aid healing
• Stop smoking, if relevant, as smoking reduces peripheral blood flow

Various drug therapies may be prescribed to enhance blood flow and/or prevent blood clotting:

• Antiplatelet agents (e.g. aspirin, dipyridamole)
• Fibrinolytic agents (e.g. danazol, tissue plasminogen activator)
• Anticoagulant agents (e.g. subcutaneous heparin injections, oral warfarin)
• Vasodilating agents (e.g. nifedipine, nicotinic acid)
• Anti-inflammatory agent (e.g. prednisone, intravenous immunoglobulin)
• Pentoxifylline (alters blood viscosity and red cell flexibility)
• Phenformin and ethyloestrenol (dissolve blood clots)

Once the condition is in remission, over time the white patches of atrophie blanche become less defined and capillaries less prominent.