Journal
JOURNAL OF DERMATOLOGICAL TREATMENT
Volume 28, Issue 6, Pages 564-566Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1080/09546634.2017.1282599
Keywords
Lupus anticoagulans; anticardiolipin; anti-beta 2-glycoprotein; digital necrosis
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Funding
- Deutsche Forschungsgemeinschaft [GO1360/4-1]
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Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disease characterized by arterial and/or venous thrombosis, recurrent abortions and detection of antiphospholipid antibodies. In fulminant cases, involvement of multiple organs can lead to significant morbidity and even fatal outcomes, so that a rapid, interdisciplinary treatment is needed. Here, we describe the case of a 39-year-old woman with a severe hard-to-treat APS with arterial occlusion and progressive skin necrosis, who was successfully treated with a combination therapy with plasmapheresis and rituximab. The treatment led to complete remission of the skin lesions for over a year. Clinical response correlated with a long-lasting reduction of antiphospholipid antibodies and B-cell depletion. This case demonstrates the use of antiphospholipid antibodies for monitoring APS-activity and shows that this severe vascular disease requires rigorous therapeutic approaches.
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