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An Unusual Case of Leg Ulceration—Diagnosis
Arch Dermatol. 2008;144(3):405-410.
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Diagnosis: Cryofibrinogenemia.
MICROSCOPIC FINDINGS AND CLINICAL COURSE
Findings from skin biopsy samples demonstrated occlusion of dermal blood vessels by eosinophilic thrombi with no evidence of vasculitis. There was necrosis of the overlying epidermis (Figure 3). Material that stained positive for periodic acid–Schiff stain was found deposited within the blood vessels. These findings were present on 4 separate biopsy samples. Test results for cryofibrinogen were positive.
Figure appears in full text version.
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Our patient was initially treated with prednisolone sodium phosphate, streptokinase, danaparoid sodium, cyclophosphamide, and stanozolol. He also had 4 sessions of plasmapheresis. These measures produced little response. After starting treatment with chlorambucil (2 mg orally for 14 d/mo) 9 months after presentation, he showed sustained improvement. To date (17 months later), he continues to receive dalteparin sodium (2500 U/d), but all other treatments have been stopped. His cryofibrinogen level, which, at its highest level was 12.5% just prior to chlorambucil treatment, fell to 4% within 5 months, and . . . [Full Text of this Article] DISCUSSION
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