The incidence in the US is reported to be 2.6 cases per 100,000 women per year.[8] Prevalence data conflict depending on the country of origin and the criteria used for diagnosis. In the US, the prevalence is thought to be 60 to 70 cases per 100,000 women,[8] affecting nearly 1 million women and men in the US and Europe.[9] Reports from Europe indicate a prevalence of 18 cases per 100,000 women, and only 3 to 4 cases per 100,000 women in northeastern Asia.[8] The disparities are influenced by the differences in diagnostic criteria based on a pathological diagnosis.

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