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Severe limb necrosis: primary thrombotic microangiopathy or “seronegative” catastrophic antiphospholipid syndrome? A diagnostic dilemma

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Abstract

An episode of gastroenteritis triggered severe necrosis of all extremities in a previously asymptomatic male. Hepatic and renal involvement were also manifest, while the hematological picture was one of thrombotic microangiopathic hemolytic anemia. Antiphospholipid antibodies were negative. He responded well to a combination of plasma exchange, anticoagulation (heparin), parenteral steroids, and antibiotics, as well as vasodilators (prostacycline) and hyperbaric oxygen, but died because of a cerebral hemorrhage. The differential diagnosis included thrombotic thrombocytopenic purpura/hemolytic–uremic syndrome, or seronegative catastrophic antiphospholipid (Asherson’s) syndrome. The dangers of administering such a combination of therapies with anticoagulation, as well as vasodilatation (prostacycline) and hyperbaric oxygen, are highlighted by the case report and emphasized.

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Aknowledgement

The evaluation of plasma ADAMTS 13 and antibodies against ADAMTS 13 were performed by Dr. Reverter and Dr. Cervera from Medical Faculty, University of Barcelona, Spain.

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Authors and Affiliations

  1. 1st Department of Internal Medicine, Medical Faculty, University Košice, tr. SNP 1, 040 11, Košice, Slovakia

    I. Lazurova, Z. Macejova, Z. Tomkova, F. Remenar, A. Boor, J. Lazur & R. Roland

  2. National Institute of Rheumatic Diseases, Piestany, Slovakia

    J. Rovensky

  3. Divsion of Immunology, School of Pathology, University of the Witwatersrand, Johannesburg, South Africa

    R. A. Asherson

Authors
  1. I. Lazurova
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  2. Z. Macejova
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  3. Z. Tomkova
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  4. F. Remenar
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  5. A. Boor
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  6. J. Lazur
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  7. R. Roland
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  8. J. Rovensky
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  9. R. A. Asherson
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Correspondence to I. Lazurova.

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Lazurova, I., Macejova, Z., Tomkova, Z. et al. Severe limb necrosis: primary thrombotic microangiopathy or “seronegative” catastrophic antiphospholipid syndrome? A diagnostic dilemma. Clin Rheumatol 26, 1737–1740 (2007). https://doi.org/10.1007/s10067-006-0487-8

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  • DOI: https://doi.org/10.1007/s10067-006-0487-8

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