Efficacité du thalidomide dans le traitement de l'histiocytose langerhansienne multisystémique de l'adulte : à propos d'une observation

RésuméIntroductionLes histiocytoses langerhansiennes multiviscérales de l’adulte sont exceptionnelles, ce qui explique que leur traitement n’est pas bien codifié.Fait cliniqueUne femme de 43 ans présentant une histiocytose langerhansienne multisystémique avec diabète insipide a été améliorée par le thalidomide 200 mg/j.DiscussionLe thalidomide a montré son efficacité de façon ponctuelle dans l’histiocytose langerhansienne avec atteinte cutanéomuqueuse mais aussi sur les lésions hypothalamiques.

BackgroundAdult multisystem Langerhans cell histiocytosis is an excepted disorder, which have several treatments. The purpose of this study was to test the disease-controlling effect of thalidomide in a case of adult multisystem Langerhans cell histiocytosis with cutaneomucous and hypothalamic localizations at dermatology department, of Mohammed-V military hospital, Rabat.Case reportA 43-year-old women, presented multifocal chronic Langerhans cell histiocytosis confined to cutaneous, oral cavity, perianal, mastoid and hypothalamic areas, with severe disabling ulcers in intertriginous areas, diabetes insipid and amenorrhoea. We treated with thalidomide 200 mg/day after neurological examination. A rapid initial response with total diminution of the involved skin area, and diminution of diabetes insipid. She remained in remission for 1 year. No adverse effects from treatment were observed at clinical and electrophysiological examinations.ConclusionThalidomide treatment is an adequate therapeutic measure in adult Langerhans cell histiocytosis, which is rare and difficult to treat. Our case showed the efficacy of thalidomide at cutaneomucosal and hypothalamic manifestations.