VPB0841 - A case of hematidrosis complicated with von Willebrand disease

Background: Hematidrosis is extremely rare disease characterized by bloody sweating from non-traumatized skin and mucous membranes. We report 14-year-old girl of hematidrosis complicated with von Willebrand disease (VWD).

Aims: To diagnose whether bleeding symptoms are due to VWD or hematidrosis.

Methods: We showed her clinical course and analyzed her blood samples.

Results: She experienced recurrent episodes of redness of eyelids and oral bleedings for one year. Her laboratory data revealed low activity of von Willebrand factor (VWF:RCo) (29 IU/dL), and low VWF antigen (46 IU/dL). The VWF multimer analysis showed all multimers were present but all were in reduced concentration. She was, therefore, diagnosed with VWD type 1. However, she experienced recurrent history of bleedings from the intact skin over the palm, face, forearms, feet, nose and mouse with no evidence of trauma at the bleeding sites, resulting in a diagnosis of hematidrosis complicated with VWD. The episodes usually happed before going to junior high school in the morning and no bleeding symptoms were seen during the holidays. Each episode lasted several hours and was usually self-limited. DDAVP (1-deamino-8-D-arginine vasopressin) was administered as a treatment for the bleedings, but no apparent effect was observed although VWF:RCo was elevated after administration of DDAVP. Comprehensive coagulation function analysis using rotational thromboelastometry (ROTEM), total thrombus-formation analysis system (T-TAS), and Multiplate were performed to evaluate her coagulation function and showed the almost normal range. Oral propranolol was commenced because the drug is considered effective for hematidrosis. No immediate effects were, however, observed after propranolol administration. She was referred to psychiatrist and her psychiatric disorders at school was revealed. The frequency of bleeding decreased remarkably after graduation form junior high school.

Conclusion(s): Her bleeding symptom was mainly because of not VWD but hematidrosis according to the data of comprehensive coagulation analysis and her clinical course.