Treatment of livedoid vasculopathy with alprostadil (PGE‐1): case report and review of published literature

Abstract

EditorThe diagnosis and treatment of livedoid vasculopathy is a challenge to the clinician. 1 A 47-year-old patient presented with livid reticular discoloration and ulceration on both legs, aggravated by exposure to cold since the age of 26 years. Clinical examination revealed painful focal purpuric ulcerating lesions, atrophic scarring, teleangiectasia, hemosiderin deposits and hyperpigmentation (Fig. 1a). Laboratory parameters such as coagulation factors, phospholipid antibodies, ANA, ENA, ANCA, C3, C4, immunocomplexes, C-reactive protein, cryoglobulins, cold agglutinins, erythrocyte sedimentation rate, serum protein electrophoresis, and urine were normal or negative. Repeated MRIs of the brain, X-rays of the chest and ultrasound investigations of the abdomen were normal. A skin biopsy specimen revealed dermal thickened vessel walls and fibrin deposits with occasional obliteration. Since 1983

Analysis of the German DRG data for livedoid vasculopathy and calciphylaxis

“…Application of low molecular heparins as non‐licensed treatment option is regularly used, but modern anticoagulants as well demonstrated effectiveness in recently performed clinical trials . In addition, vasodilators like prostaglandins might be successfully used for treatment of LV to promote healing of ulcerations . According to the literature, women are more often affected with a ratio of women:men up to 3:1 .…”

Treatment of Skin Ulcers Secondary to Sneddon Syndrome With Alprostadil (Prostaglandin E1)

“…This is a case of a patient with TEC with unilateral sparing of an axilla that had previously been exposed to radiation therapy and ALND. 4 Though this patient reported little baseline sweating, and therefore did not note hypohidrosis in the left axilla, decreased sweat production in the distribution of the intercostobrachial nerve is a complication of mastectomy and ALND. Sparing of an area of sympathetic denervation in this patient supports the theory that TEC is caused by excretion of chemotherapeutic agents in sweat.…”

“…Mittag et al 3 reported the first case of SS treated with iloprost in cycles also obtaining a remission in pain and clearing of the skin ulcers. Mofarrah et al 4 described a case of livedoid vasculitis successfully treated with PGE-1 at dose of 60 μg for 5 days followed by a dose of 60 μg of monthly maintenance. The pain disappeared in the first 2 days, and ulcers healed after 3 weeks of treatment.…”

“…Alprostadil seems to be more effective in occlusive vasculopathy with racemous livedo. In the case reported by Mofarrah et al, 4 iloprost was not as effective as Prostanoids have also been used in patients with peripheral arterial disease (PAD) in association with revascularization to relieve pain or improve ulcer healing. 5 Prostanoids are probably not as effective in treating PAD because this conditions it is not an alteration of microcirculation as in livedoid vasculitis and SS.…”

Effect of alprostadil on erythrocyte deformability in ischemia reperfusion injury

“…Prostaglandin E1 (PGE 1 ) has protective effects against acute and chronic ischemia in various organs (9)(10)(11)(12)(13)(14).…”