Catastrophic antiphospholipid syndrome: A case with unusual findings revealed in autopsy and a brief literature update

Signorelli, Flávio; Balbi, Gustavo Guimarães Moreira; Levy, Roger A.

doi:10.5152/eurjrheum.2017.17023

Cited by 1 publication

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References 7 publications

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“…Most episodes are precipitated by infections, surgery, or malignant diseases. In a majority of cases, small vessels are affected leading to a disseminated microangiopathic syndrome resembling thrombotic thrombocytopenic purpura [ 21 ]. The most frequently involved sites are kidney (73%), lungs (60%), brain (56%), and heart (50%) [ 22 ].…”

Section: Discussionmentioning

confidence: 99%

Extensive abdominal wall ulceration as a late manifestation of antiphospholipid syndrome: a case report

2018

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BackgroundAntiphospholipid syndrome is an autoimmune disorder characterized by the presence of antiphospholipid antibodies and commonly presents with vascular thromboembolic phenomena, thrombocytopenia, and obstetric complications. Antiphospholipid syndrome can be classified as either primary or secondary to other connective tissue diseases. Dermatologic manifestations are common; however, non-vasculitic skin ulceration is an uncommon manifestation of antiphospholipid syndrome with limited treatment options.Case presentationIn this paper we report the case of a 58-year-old white woman who developed necrotic abdominal wall ulcers 27 years after a diagnosis of secondary antiphospholipid syndrome associated with systemic lupus erythematosus. The ulcers developed despite our patient being on therapeutic anticoagulation with warfarin and were resistant to further increases in the intensity of anticoagulation. Management was further complicated due to reluctance on the part of our patient to switch over to injectable heparin.ConclusionsThis case highlights a rare late dermatologic presentation of antiphospholipid syndrome, which responded poorly to conventional anticoagulation with warfarin. Current management is limited to experimental therapies and the role of newer anticoagulants is still unknown.

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Section: Discussionmentioning

confidence: 99%