Effectivement, il faut penser au Fish Odor Syndrome ou triméthylaminurie [URL="http://escholarship.org/uc/item/9550x15j#page-2"]Fish odor syndrome: a case report of trimethylaminuria[/URL]
Les auteurs de cet article recommandent des lavages fréquents et l'utilisation de déodorants, un régime pauvre en choline, lécithine et carnithine ( sans poissons, foie, oeuf, soja, légumes, lait) et en cas d'échec la prescription de métronidazole.
Trimethylaminuria is a rare, autosomal recessive, metabolic disorder that results in accumulation of trimethylamine (TMA), which smells like rotten fish. The chemical is excreted in sweat and urine owing to a deficiency in the enzyme flavin monooxygenase 3 (FMO3). We report a case of trimethylaminuria in a 12-year-old girl. The patient failed treatment with diet and hygiene modification, but achieved symptomatic improvement after a four-month course of metronidazole.
Trimethylaminuria is a rare metabolic disorder resulting in accumulation of trimethylamine (TMA) in sweat and urine owing to enzymatic deficiency of flavin monooxygenase 3 (FMO3) caused by mutations in the FMO3 gene. Primary trimethylaminuria has an autosomal recessive inheritance pattern [1]. Normally, hepatic FMO3 oxidizes TMA, which smells like rotting fish, into trimethylamine-N-oxide (TMAO), which is odorless 1]. In trimethylaminuria, TMA is excreted through the sweat and urine, resulting in a characteristic fishy body odor [2]. Increased sweating related to exercise or anxiety exacerbates the odor [3].
Initial treatment includes restriction from foods containing choline, lecithin, and carnitine (saltwater fish, liver, egg yolk, soy beans, and legumes), which are precursors to TMA [1, 2]. The excessive consumption of milk has also been linked to increased TMA excretion [4]. Even with rigorous adherence to the restricted diet, the amount of TMA excreted fluctuates [3]. Frequent bathing and deodorants may help conceal the odor [5].
If clinical improvement is not accomplished through diet alone, metronidazole may be used to decrease microbial gut flora and reduce TMA production [2, 3, 5]. Neomycin, activated charcoal, and lactulose have been used in the past, but frequently cause diarrhea. To prevent treatment failure related to drug resistance, the administration of metronidazole for one- to two-week intervals interspersed with drug-free periods is advised [3].
Dernière modification par dermatologue ; 11/09/2014 à 08h16.
L'information ou le conseil en ligne ne sont pas des consultations médicales et ne doivent exonérer ni le médecin ni le patient d'une véritable consultation avec une anamnèse et un examen clinique qui seuls peuvent permettre d'aboutir à un diagnostic et à une prescription.
Dr Philippe Abimelec, Dermatologue
Répondre avec citation
