[Essential cryofibrinogenemia with generalized livedo racemosa].
Copyright policy )[Essential cryofibrinogenemia with generalized livedo racemosa].
A 45-year-old male patient presented with cold-induced generalized livedo reticularis, repeated acral ulcerations and purpura, Raynaud's phenomenon, and peripheral polyneuropathy. The patient also experienced malaise, vertigo, and transient amaurosis whenever he was exposed to low temperatures. Skin biopsies of the livedo reticularis revealed marked dilatation of the small dermal blood vessels and circumscribed leukocytoclastic vasculitis. Essential cryofibrinogenaemia was diagnosed on the basis of detection of this cryoprotein in the chilled blood plasma with increased viscosity. Pulsed therapy with dexamethasone and cyclophosphamide resulted in marked relief of the symptoms, and cryofibrinogen was no longer detectable in the patient's plasma.
- University of Münster Germany
Male, Vasculitis, Biopsy, Fibrinogens, Abnormal, Fibrinogen, Fluorescent Antibody Technique, Raynaud Disease, Middle Aged, Dexamethasone, Drug Administration Schedule, Capillaries, Humans, Drug Therapy, Combination, Cyclophosphamide, Foot Ulcer, Cryoglobulins, Skin
Male, Vasculitis, Biopsy, Fibrinogens, Abnormal, Fibrinogen, Fluorescent Antibody Technique, Raynaud Disease, Middle Aged, Dexamethasone, Drug Administration Schedule, Capillaries, Humans, Drug Therapy, Combination, Cyclophosphamide, Foot Ulcer, Cryoglobulins, Skin
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