Oral pyogenic granuloma arising in von willebrand disease setting : an atypical case report

Background: At times, behind a usual picture of sub-mucous reactive lesion such as oral pyogenic granuloma (OPG) it may hide an often misdiagnosed underlying coagulation disorder. Describing this report we seek to suggest a rational diagnostic approach and surgical management of an OPG in von Willebrand disease patient (VWD). Methods: In April 2009, a 75-years-old patient presented to our department with a history of bacterial gingivitis and consequent gingival bleeding. Oral examination displayed a nodular lesion on the superior-anterior gingival mucosa, 1.5 per 1 cm, clinically compatible us a pyogenic granulomas picture. The patient anamnesis was positive for episodic epistaxis and ecchymosis. Considering the bleeding tendency of patients we required an initial laboratory evaluation, including the bleeding time (BT), the prothrombin time (PT), activated partial thromboplastin time (APTT), and thrombin time (TT). The blood test findings showed a prolonged APTT and BT, which justified an additional laboratory evaluation for diagnosing or excluding factor-VIII abnormality. We programmed the measure of the VWF protein amount present in the plasma (VWF:Ag), the function of the VWF protein that is present as ristocetin cofactor activity (VWF:RCo) and factor VIII procoagulant activity (FVIII:C). FVIII:C levels were low but measurable, VWF:RCo and VWF:Ag showed a relative decrease. Thus, The patient was referred to the Haematology Center of our Hospital for additional tests In order to prevent intra and post-surgical bleedings, as a first therapeutic approach, the patient was treated by 1-Deamino-8-d-arginine-vasopressin (DDAVP) infused IV at a dose of 0,3 μg/kg without significative BT and FVIII/vWF improve. Consequently, he was treated with FVIII/vWF concentrate infusion at a daily dose of 40 UI/Kg until third post-operative day, achieving a BT shortened and a transitory FVIII/vWF correction. To increase antifibrinolytc activity and to suppress hyperfibrinolysis in oral tissues, were required to rinse with 10 mL of a 4.8% tranexamic acid solution 4 times at day for 7 days postoperatively. The lesion excision was carried out by diode laser (wavelength, 808 nm) set at 1,5 W in a pulsated wave mode, suturing the depth area in connective tissue. Discussion: At present, no consensus exists on the therapeutic management of VWD, especially in relation to oral soft tissues surgery. Conclusions: As some VWD patients can be unresponsive to DDAVP, local gingival haemostasis may benefit 40-60 U (as VWF:RCof)/kg of a FVIII/VWF concentrate. In addition, oral administration of 20 mg/kg/day of tranexamic acid should be combined with the regimens described above.