On admission multiple livedoid erythematous–violaceous plaques were present on her lower abdomen, thighs and buttocks (Fig. 1). The patient had no fever. Laboratory tests showed an increased leukocyte count (14.5… Click to show full abstract
On admission multiple livedoid erythematous–violaceous plaques were present on her lower abdomen, thighs and buttocks (Fig. 1). The patient had no fever. Laboratory tests showed an increased leukocyte count (14.5 × 109/L) without eosinophilia and raised C-reactive protein levels (14.5 mg/dL). A complete autoimmune evaluation (antinuclear antibodies, antimitochondrial antibodies, extractable nuclear antigen antibodies, complements C3 and C4 levels, antineutrophil cytoplasmic antibodies, anti-proteinase 3 and anti-myeloperoxidase antibodies, complement C1q immune complex) disclosed no abnormalities. No cryoglobulins were found. Proteins S and C activity levels were, respectively, 82% (normal values >65%) and 68% (normal values >75%). No bacteriological culture was obtained. An incisional biopsy specimen of the thighs revealed necrosis of the vasal wall, intraluminal thrombi and diffused interstitial neutrophilic infiltrate; no evidence of cholesterol emboli. Unfortunately, no markers for endothelial proliferation were tested. Steroid therapy (metilprednisolone) was prescribed and therapy with warfarin stopped (in the suspect of warfarin induced cutaneous necrosis), without any improvement. The lesions evolved quite rapidly (14 days) into more extended, painful, necrotic and ulcerated plaques (Figs. 2, 3, 4). After a few days the patient was found dead in her bed. The autopsy revealed severe diffuse aortosclerosis with a complete occlusion of both external iliac arteries, bilateral nephroangiosclerosis and posterior myocardial infarction with severe coronarosclerosis.
               
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