[Acquired von Willebrand disease in a patient with urticarial vasculitis and myomatouse uterus].

We describe a 51-year old-woman with haemorrhagic syndrome manifested as spontaneous bruising, and prolonged and abundant menstrual bleeding. She had hed these symptomes for six months prior to admission, but denied lifelong bleeding tendency. Gynaecological examination revealed a myomatouse uterus. Occasionally, the patient had crops of erythemo-exudative skin lesion which were shown on biopsy to represent urticarial (leukocytoclastic) vasculitis. No evidence of systemic connective tissue disease was found. Haemostasis testing revealed: prolonged bleeding time, decreased platelet adhesiveness to glass beads, lowered FVIII : C, absence of ristocetin-induced platelet aggregation and unmeasurable FVIII : Af and vWf. There was no evidence for an inhibitor to FVIII : C. However, patient's plasma, when mixed ana partes equales with normal plasma, reduced the FVIII : Ag and vWf level to 48% and 46% respectively, as compared to the mixture of normal plasma and buffer. When incubated at room temperature, patient's washed platelets spontaneously released vWf and aggregated in the presence ristocetin. However, when patient's plasma was added aggregation was inhibited. On the basis of these findings and the lack of anamnestic data suggestive of congenital haemorrhagic disorder, we concluded that the patient had acquired von Willebrand disease due to an inhibitor to vWf and FVIII : Ag, associated with urticarial vasculitis and myomatouse uterus. To our knowledge, this association has not yet been reported.