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Le syndrome BASCULE : une étude rétrospective montpelliéraine de 45 cas
Mémoire de Master / Thèse d'exercice   Open Access

Le syndrome BASCULE : une étude rétrospective montpelliéraine de 45 cas

Céline Devoye
Masters , Université de Montpellier
13/10/2023

Résumé

BASCULE syndrome Autonomic dysfunction Bier Anemic Spots Cyanosis with Urticaria-Like Eruption Syndrome BASCULE Dysfonctionnement autonome Postural orthostatic tachycardia syndrome Orthostatic intolerance Hypotension, orthostatic Syndrome de tachycardie orthostatique posturale Intolérance orthostatique Hypotension orthostatique
BASCULE syndrome (Bier Anaemic Spots, Cyanosis, Urticaria-Like Eruption) is a relatively unknown benign vasomotor dermatosis primarily affecting infants and adolescents.An association between this syndrome and dysautonomia or other cardiovascular comorbidities in adolescents has been frequently mentioned in the literature. The aim of this study was to gain a better understanding of this syndrome through the description of a Montpellier cohort and to assess the necessity of cardiovascular exploration.Method: a retrospective study was conducted on patients diagnosed with the BASCULE syndrome from January 2016 to July 2023 at the University Hospital of Montpellier. Results: we diagnosed 45 patients with BASCULE syndrome, including 12 infants and 33 older children, adolescents, and adults. In the infant group, all cardiovascular explorations were normal, and the outcome showed spontaneous improvement. In the other group, the median age of symptom onset was 13 years. The eruption was predominantly bilateral, and accompanying signs and symptoms were present in 85% of patients. Dysautonomia and non-dysautonomic cardiovascular comorbidities were present in 13% and 18% of patients, respectively. Conclusion: BASCULE syndrome manifests in various clinical forms: (i) in infants, where the syndrome appears to be isolated and spontaneously resolves with the acquisition of walking; (ii) in older children, adolescents, and adults, which should prompt screening for dysautonomia; and (iii) as a locoregional form of BASCULE syndrome secondary to peripheral neuropathy.

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