Sneddon syndrome (SS) is a rare noninflammatory systemic vascular disease that clinically presents with cerebrovascular disease and racemous livedo.1 This condition is characterized by obstructive vasculopathy of cutaneous microcirculation, which can lead to the development of painful skin ulcers.
A woman in her 50s with a history of SS of 20 years’ evolution came to the dermatology department with ulceration on both legs. The patient had a history of lacunar infarction and was taking acetylsalicylic acid for prevention of cerebral ischemic events. Skin ulcers were previously treated with systemic corticosteroids and clopidogrel with no response. Physical examination revealed the presence of ulcerations of an irregular shape 1 to 5 cm in diameter with retiform purpura in both lower limbs (Figure, A). In addition, racemous livedo was observed on almost all the body surface. Peripheral pulses in upper and lower limbs were preserved bilaterally. No signs of severe chronic venous insufficiency or palpable purpura were observed. Laboratory findings for differential diagnosis of SS were normal or negative. Dermatopathologic analysis showed presence of fibrin thrombi and wall thickening in the papillary dermis with neovascularization phenomena. No fibrinoid necrosis of the vessel wall, neutrophilic infiltrate, or leukocytoclasia was observed. A diagnosis of SS was confirmed.
Figure. The Leg of a Patient With Sneddon Syndrome
A, Before treatment, the leg shows multiple ulcerations. B, The ulcerations are cleared after 3 months of alprostadil treatment.
Treatment with intravenous alprostadil (prostaglandin E1 [PGE-1]) (Prostavasin; Gebro Pharma GmbH) was started at doses of 60 µg every 24 hours for 5 days and then a dose of 60 µg every 24 hours monthly as maintenance. From the first infusion, the patient showed rapid improvement in pain. After 3 months of treatment, complete healing of the skin ulcers was observed (Figure, B). At last follow-up, the patient had been treated for 6 months with a monthly infusion of alprostadil and remained asymptomatic. No adverse effects were observed.
There are no established guidelines regarding the treatment of SS. Steroids and immunosuppressants have been used, but their effectiveness is still controversial, given the absence of vascular inflammation. Anticoagulant and antiplatelet agents have been used for the long-term prevention of cerebral ischemic events. Antiplatelets are more suitable when findings of antiphospholipid antibodies (aPL) are negative; anticoagulants are more suitable when aPL findings are positive.2
Different therapies such as intravenous immunoglobulins, rivaroxaban, nifedipine, or those similar to prostaglandin have been used to treat the cutaneous symptoms. Mittag et al3 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 al4 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.
Both livedoid vasculitis and SS are cutaneous microcirculation abnormalities characterized by obliterative phenomena in the vessels. Noting this similarity, we used the same treatment regimen for SS. 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 alprostadil. Alprostadil exerts a vasodilator, antiplatelet, and cytoprotective effect; it is an inhibitor of smooth muscle proliferation and fibrinolytic activity. Potential adverse events can occur, including fever, flushing, hypotension, hypocalcemia, and apnea.
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. In venous ulcers, PGE-1 has also been described to be effective.6
We describe the successful treatment of a case of SS with alprostadil. This drug induces immediate pain relief, complete healing, and prevention of new skin ulcers. Alprostadil may be a therapeutic alternative for other dermatological conditions secondary to obstructive vasculopathy of cutaneous microcirculation.
Corresponding Author: Cristina Collantes-Rodríguez, MD, Unidad de Gestión Clínica de Dermatología, Hospital Universitario Puerta del Mar, Avenida Ana de Viya 40 nº 21 CP:10009, Cádiz, Spain (firstname.lastname@example.org).
Published Online: March 16, 2016. doi:10.1001/jamadermatol.2016.0162.
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information. We are also indebted to Karla Tello-Collantes, MD, Department of Anatomic Pathology, Hospital Universitario Puerta del Mar, Cádiz, Spain, who provided expertise in pathology. She received no compensation for her contributions.
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