By Gary M. White, MD
Trimethylaminuria, AKA Fish Odor Syndrome, is a rare autosomal recessive disorder of metabolism caused by a mutation in flavin-containing monooxygenase 3 (FMO3 gene).
- The patient gives off a strong fishy odor (like dead fish) or strong body odor (like rotten eggs).
- Onset is often in adolescence.
- Trimethylamine is present in the person's sweat, urine, and breath causing the odor.
- It is diagnosed by urine analysis for the ratio of trimethylamine to trimethylamine oxide.
- Expression is variable and only some members of a family may manifest the odor.
- Sometimes dermatologists consider this condition in the differential of bromhidrosis.
Genetic counseling is in order. Referral to a dietician for aid in minimizing consumption of certain foods, e.g., eggs, legumes, fish, and certain meats is recommended. Daily or twice a day shower, using deodorants, and avoiding hot, sweaty environments is helpful. Oral metronidazole can reduce gut flora and be very helpful [Dermatology Online Journal 20(1)].
Psychological counseling may be needed.
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