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Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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It is a comparatively rare disease with an incidence between 11.5 and 75.0 per 100 000 population in the northern Europe

  • in Scotland - 11.5 per 100,000
  • in Sweden - ranging from 19.6 to 39.2 per 100,000

The condition usually presents in the fourth and fifth decades (but may present at any age).

Men are affected more frequently than women (two thirds of patients are male) (3)

Caucasians are affected predominantly (1).


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