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Venous thromboembolism (VTE) has been reported to be a significant cause of death in patients with pemphigus.We sought to assess the incidence and characteristics of VTE in patients with pemphigus.A retrospective study following a cohort of 172 patients with newly diagnosed pemphigus for the development of VTE was conducted.Over a mean follow-up time of 4 years, 10 patients (6%) had development of VTE at a median of 4 months from pemphigus diagnosis. The highest risk was found in the first year, at 5 VTE events per 100 patient-years or a 5% VTE risk for that first year. Five patients had deep vein thromboses, 4 had pulmonary embolisms, and 1 had both. Age and gender were not related to the VTE risk. Most patients had VTE risk factors, either hereditary or acquired as the result of pemphigus treatments and their complications, including hospitalization, immobilization, and infections.The use of existing health records limited the assessment of asymptomatic VTE and VTE risk factors; a matched control population was not studied.VTEs are not rare in patients with pemphigus, more so in the first year after diagnosis. VTE risk must be assessed in all patients, especially when hospitalized, and thrombo-prophylaxis should be initiated in qualifying cases.