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  Authority of Polish Society of Allergology

vol 16. no 1. March 2011  

 Original articles
Extensive skin necrosis in a patient with antiphospholipid syndrome and hemolytic anemia
Weronika Kleczyńska, Aleksandra Matyja-Bednarczyk, Grzegorz Dyduch, Jacek Musiał

Antiphospholipid syndrome (APS) is an autoimmune disorder manifesting
itself with arterial and/or venous thrombosis and pregnancy
morbidity associated with antiphospholipid antibodies. Skin manifestations
of APS may take different forms. Repeatedly, the coexistence
of APS and systemic vasculitides have been suggested, but
the basis of such correlation remains unclear. The case report presented
herein, refers to a female patient with heamolytic anemia,
extensive skin necrosis due to the antiphospholipid syndrome, and
the suspicion of microscopic vasculitis. Laboratory tests showed the
presence of lupus anticoagulant, elevated anticardiolipin antibodies
in both classes (IgG and IgM) and IgM class of anti-β2-glycoprotein
antibodies. Skin biopsy revealed thrombi in the superficial vessel
plexus with immunological deposits in the vessel walls. There were
no findings characteristic for vasculitis. Based on the clinical picture
and the laboratory findings the antiphospholipid syndrome was
diagnosed. Due to the presence of anti-MPO a microscopic vasculitic
component could not have been excluded. Therapy included
low-molecule heparin, methylprednisolon and erythropoietin (no
patient consent to blood substitute treatment due to her being
a member of Jehovah’s Witnesses). In the place of massive skin effusions,
dry and subsequently colliquative necrosis developed. The
patient consistently did not agree to proposed surgical treatment
and skin transplant. She chose alternative treatment (ozonotherapy),
which caused complications: Pseudomonas aeruginosa sepsis,
acute renal failure, ischemic stroke, lower gastrointestinal tract
bleeding, disseminated intravascular coagulation and finally death.
The following case report displays difficulties in the differential diagnosis
of autoimmune disorders.

keywords: zespół antyfosfolipidowy, toczeń rumieniowaty układowy, martwica skóry, niedokrwistość hemolityczna, antiphospholipid syndrome, systemic lupus erythematosus, skin necrosis, hemolytic anemia

pages: from 44 to 48

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