Dermatoneuro syndrome au cours d’un scléromyxœdème : efficacité des plasmaphérèses et des immunoglobulines intraveineuses

Introduction Le dermatoneuro syndrome est une complication neurologique particuliere du scleromyx�deme, rapportee une vingtaine de fois dans la litterature medicale. Il associe fievre, coma, convulsions et syndrome pseudo-grippal. Le devenir des patients atteints est mal connu. Nous decrivons l�observation d�une patiente ayant repondu favorablement a l�association de plasmaphereses et d�immunoglobulines intraveineuses (IgIV). Observation Une femme de 57 ans etait atteinte de scleromyx�deme evoluant depuis 14 ans, resistant a de multiples therapeutiques. En novembre 2011, elle presentait un premier episode de crises convulsives suivi d�un coma post-critique, puis d�un syndrome confusionnel associe a des hallucinations visuelles, spontanement resolutif en quelques jours. La recherche d�une infection etait negative et l�imagerie cerebrale (tomodensitometrie et IRM) ainsi que l�electroencephalogramme etaient parfaitement normaux, soulevant l�hypothese d�une atteinte neurologique de son scleromyx�deme. En decembre 2012 et aout 2013, elle presentait deux nouveaux episodes d�etat de mal epileptique, suivis a nouveau d�un etat confusionnel, pour lequel la recherche etiologique etait encore negative. Cette fois, l�etat confusionnel se perennisait pendant deux mois, jusqu�a la mise en place des echanges plasmatiques, suivie d�une cure d�IgIV. Cette association therapeutique permettait d�obtenir une regression rapide de l�ensemble des symptomes neurologiques et une amelioration de l�etat general. Discussion Le dermatoneuro syndrome est une complication neurologique centrale rare du scleromyx�deme. Sa physiopathologie est inconnue. La gammapathie monoclonale associee au scleromyx�deme pourrait expliquer un etat d�hypercoagulabilite ou la formation d�agregats neutrophiliques aboutissant a une alteration de la microcirculation. Le traitement est empirique et mal codifie. L�evolution est imprevisible et potentiellement letale. Summary Background Dermato-neuro syndrome is a specific neurological complication of scleromyxedema presenting with fever, coma, seizures and flu-like syndrome. To our knowledge, it has only been reported about twenty times in the literature. Its outcome is uncertain. We describe the case of a patient in whom a favorable outcome was achieved using a combination of plasmapheresis and intravenous immunoglobulin (IVIG). Patients and methods A 57-year-old woman was diagnosed 14 years ago with scleromyxedema resistant to multiple lines of treatment. In November 2011, she presented an initial episode of epileptic seizure followed by post-seizure coma, and later, confusional state with visual hallucinations. She recovered spontaneously within a few days. CT scan, MRI, EEG and screening for infection were perfectly normal, resulting in suspicion of neurological involvement associated with her scleromyxedema. In December 2012 and August 2013, she presented two further episodes of status epilepticus, followed once more by a confusional state, with etiological explorations again proving unfruitful. On this occasion, her confusional state persisted for two months until the initiation of plasmapheresis and IVIG. This combination therapy led to rapid regression of all neurological symptoms and an improvement in her general condition. Discussion The dermato-neuro syndrome is a rare neurological complication of scleromyxedema. Its pathophysiology is unknown. The monoclonal gammopathy induced by the scleromyxedema could account for the patient's hypercoagulable state and for the formation of neutrophilic aggregates leading to impaired microcirculation. Treatment is empirical and poorly codified. The course of the disease is unpredictable and may be lethal.