Dermatoses are often the first sign of APS.
Early recognition of these skin diseases as manifestations of antiphospholipid syndrome (APS) may enable providers to forestall a primary thrombotic event or miscarriage, said Camille Frances, M.D., of the Hopital de la Pitie in Paris.
In a study of 100 patients with primary APS and 100 with systemic lupus erythematosus (SLE)-related APS, dermatologic manifestations were often the first clinical sign, Dr. Frances reported at an international conference on cutaneous lupus erythematosus.
Her study is believed to be the first dermatologic assessment of the scope of skin manifestations in APS. The study included only patients whose disease fulfilled the clinical and laboratory criteria for APS.
The dermatologic conditions included livedo reticularis, skin ulcerations, pseudovasculitis lesions, superficial skin necrosis, digital gangrenes, superficial phlebitis, multiple subungual splinter hemorrhages, and anetoderma.
Originally, 36 of the primary APS patients and 25 of those with SLE-related APS appeared with a dermatologic problem. During the follow-up of at least 5 years, 45 of the primary APS and 53 of the SLE-related APS patients developed a dermatologic manifestation of APS.
Livedo reticularis, for which there is no specific therapy, was the most common skin manifestation, with 36 of the 200 patients appearing with it and 51 of the patients showing it by the end of follow-up. These lesions persisted "despite frequent fluctuations," lacked infiltration of the reticular pattern, and were either widespread or localized on the limbs or on the trunk or buttocks--sometimes in both areas. Lesions had an irregular fishnet reticular pattern that lacked infiltration.
Patients who developed livedo reticularis were significantly more likely than those without such lesions to have seizures, Raynaud's phenomenon, arterial events (cerebral or ocular ischemic events in particular), systemic hypertension (higher than 160/90 mm Hg), and heart valve abnormalities as revealed on echocardiography.
On the other hand, livedo reticularis patients were significantly less likely to have venous thrombosis alone than patients without the skin condition.
No obvious pathological changes could be detected in 19 of 26 patients (15 primary, 11 SLE-related) with livedo reticularis who underwent two skin biopsies each "despite a very uniform standardization of the biopsies," Dr. Frances said. The other patients showed vascular abnormalities in small- to medium-sized arteries.
Necrosis of the digits was the initial manifestation in 5 patients and eventually developed in a total of 15.
A Doppler echo or angiography revealed occluded arteries as the cause of necrosis. Four patients required amputation of one or two digits. Four patients had extensive cutaneous necrosis that appeared similar to necrotic lesions in other thrombophilic states.
For those who develop cutaneous necrosis, Dr. Frances advised keeping patients fully anticoagulated with heparin. When this does not stop the extension of necrosis, she suggested using iloprost (an intravenous form of prostacyclin that imitates natural prostaglandin) or plasma exchanges plus steroids or cytotoxic agents or both.
Multiple subungual splinter hemorrhages developed alongside arterial events in some patients. Originally, the hemorrhages were seen as the first skin manifestation in 4 patients and later occurred in 10 patients. Livedoid vasculitis-like ulcers were an initial skin sign in four patients. Nine women overall had a recurrence of postphlebitic ulcers, one of whom first presented with the skin problem.
Four patients were seen with pseudovasculitis lesions (purpura, palmar or plantar erythema, nodules, pustules, and atrophic lesions), and that group expanded later to six patients.
Circumscribed skin necrosis and pseudovasculitis lesions need to be treated with antiplatelet agents such as the combination of low-dose aspirin and dipyridamole (Aggrenox), anticoagulants, and fibrinolytic agents, she added.
BY JEFF EVANS
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|Title Annotation:||Dermatology; Antiphospholipid syndrome|
|Publication:||Internal Medicine News|
|Date:||Dec 1, 2004|
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