[Scleromyxedema with laryngeal changes. Beneficial results of the treatment with mucopolysaccharidases].
We observed a case of scleromyxoedema with specific cutaneous lesions in a 62-year-old man. These lesions were associated with modifications of the central nervous system, latent thyroid failure, cardiovascular changes and myopathy, usual manifestations of this disease, but without paraproteinaemia. Unusual laryngeal manifestations were a dysphonia with chronic pseudomyxomatosis of the larynx. Urinary mucopolysaccharide excretion was increased. It is important to underscore the favourable response to Thiomucase when more toxic drugs had become ineffective.
Clinique Dermatologique, Université de Médecine Cluj-Napoca, Romania.
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R
Cosgarea
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M
Cosgarea
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T
Turcu
D005966:Glucuronidase / Q000627:therapeutic use; D006801:Humans; D007818:Laryngeal Diseases / Q000209:etiology*; D008190:Lyases / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009230:Myxedema / Q000150:complications* / Q000188:drug therapy; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy
C021812:mucopolysaccharidase; D005966:Glucuronidase; D008190:Lyases
Scléromyxoedème avec modifications laryngées. Résultats bénéfiques du traitement par mucopolysaccharidases.