Sudden death in urticaria pigmentosa

A case of sudden death of a 25-year-old man, with a six years history of Urticaria pigmentosa, is reported. On a cold morning in November he was found lying dead on the lawn. Histology revealed no signs of systemic mastocytosis. The biochemical investigations (high free histamine level in urine) in addition to a BAC of 0,72 mg/g and the scene conditions (cold irritation) explained the mechanism of death, a systemic anaphylactoid reaction.