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A Genetic Origin? Purpura Fulminans

The patient also had hemorrhagic necrosis of the skin and soft tissue of the right thigh.

Over days, a patient’s relatively small lesion evolved into widespread tissue damage. A previously healthy 49-year-old Arab woman was admitted for a 2-cm painful red lesion on her left breast. She had been well until a few days earlier, when she developed left flank pain and was treated with ofloxacin for a presumed urinary tract infection. During the first 48 hours of her admission, the erythematous lesion over the left breast progressed, developing the appearance of a hematoma. Ultimately, it involved the entire breast (Figure 1). The patient denied trauma. At the same time, her hemoglobin level decreased by 2 g/dL. Her prothrombin time, partial thromboplastin time, fibrinogen concentration, and platelet count were all within normal limits.



Over the following 24 hours, the patient’s skin and underlying breast tissue became necrotic. Three days later, a simple mastectomy was performed. The next day, the same process began on the right breast. Given the clinical picture and the rapid and systemic nature of the process, methylprednisolone was administered to treat possible vasculitis. Heparin treatment was considered but withheld because of the hemorrhagic appearance of the breast. Nonetheless, necrosis of the skin overlying the breast progressed and was associated with severe pain. A second simple mastectomy was performed 2 days later. Pathologic specimens obtained from the breasts revealed hemorrhagic necrosis, microvascular occlusion, and an acute inflammatory infiltrate, but no evidence of vasculitis.

Laboratory investigations excluded the presence of antibodies associated with heparin-induced thrombocytopenia, antiphospholipid antibodies, and lupus anticoagulant activity. An antinuclear antibody test, positive at a titer of 1:80, was of borderline significance. The patient was also found to be homozygous for the Factor V Leiden mutation. Protein S activity was reduced at 32% (normal, >55%).

A week later, the identical process of hematoma formation followed by frank hemorrhagic necrosis developed over the patient’s right thigh (Figure 2). A biopsy of the edge of the lesion showed microscopic signs of leukocytoclastic vasculitis.


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-Orly Avnery, MDa,b, Gili Kenet, MDb,c, Martin H. Ellis, MDa,b

-This article originally appeared in the March issue of The American Journal of Medicine.

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