[Sudden death in the presence of systemic mastocytosis and urticaria pigmentosa]

Abstract

Systemic mastocytosis is an extremely rare disease characterised by abnormal mast cell production and accumulation of mast cells in the bone marrow or organs, and to some extent also in the skin (urticaria pigmentosa). To date there have been no descriptions of death caused by systemic mastocytosis. The present first description of such a fatality is based upon a case of anaphylactic histamine shock, probably caused by the consumption of peanuts. The affected person suffered from urticaria pigmentosa and developed a fairly typical pruritus prior to death. In the serum sample taken post-mortem the tryptase concentration was markedly elevated. The diagnosis of systemic mastocytosis was established on the basis of two primary and three secondary criteria according to the WHO classification, which underlines the importance of histological investigations in cases where the cause of death is unclear.