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[Sudden death in urticaria pigmentosa]
J Bolt1, B Schellmann, O Braun-Falco
1Institut für Rechtsmedizin, Universität Erlangen-Nürnberg, Bundesrepublik Deutschland.
Zeitschrift Fur Rechtsmedizin. Journal of Legal Medicine
|January 1, 1990
Summary
Sudden death in a young man with Urticaria pigmentosa was linked to a systemic anaphylactoid reaction. Cold exposure and elevated histamine levels, not systemic mastocytosis, were key factors.
Area of Science:
- Forensic Pathology
- Toxicology
- Dermatology
Background:
- Urticaria pigmentosa (UP) is a rare skin condition characterized by mast cell infiltration.
- Systemic mastocytosis (SM) can occur in association with UP, potentially leading to severe complications.
- Sudden death in individuals with UP necessitates thorough investigation into potential underlying causes.
Observation:
- A 25-year-old male with a 6-year history of Urticaria pigmentosa was found deceased outdoors on a cold morning.
- Autopsy and histological examination did not reveal evidence of systemic mastocytosis.
- Biochemical analysis showed elevated urinary histamine levels and a blood alcohol content (BAC) of 0.72 mg/g.
Findings:
- The cause of death was determined to be a systemic anaphylactoid reaction.
- The reaction was likely triggered by environmental cold exposure, exacerbating the underlying mast cell disorder.
- High free histamine levels and alcohol intoxication contributed to the fatal outcome.
Implications:
- This case highlights the potential for severe, life-threatening anaphylactoid reactions in individuals with Urticaria pigmentosa, even without overt systemic mastocytosis.
- Environmental triggers, such as cold, can precipitate fatal reactions in susceptible individuals.
- The findings underscore the importance of comprehensive toxicological and biochemical investigations in sudden death cases associated with chronic mast cell disorders.