BASCULE syndrome associated with presyncopal episodes and first‐degree atrioventricular block

Guillén-Climent, Santiago; Saura, Saray Porcar; Benavent, Martí Pons; García‐Vázquez, Alejandro; Martín, J.M.

doi:10.1111/ijd.15568

Cited by 8 publications

(15 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A total of 81% of specialists of the survey offered only symptomatic pieces of advice, e.g., performing exercise and avoiding prolonged orthostatism. Based on the hypothesis, the causative mechanisms are the mast cell degranulation, and treatment with anti-H1 antihistaminics, such as desloratadine, cetirizine, or bilastine, has been proposed in the literature ( 8 , 9 ). They were applied in 33% of patients reported in the literature (7 of 21 including our case) and by 11% of physicians of our survey but proved ineffective in most cases.…”

Section: Discussionmentioning

confidence: 99%

Case Report, Practices Survey and Literature Review of an Under-Recognized Pediatric Vascular Disorder: The BASCULE Syndrome

et al. 2022

View full text Add to dashboard Cite

IntroductionBier anemic spots, cyanosis, and urticaria-like eruption (BASCULE) syndrome is an underreported pediatric vascular disorder from the group of acrosyndromes. In children, these include paroxysmal acrosyndromes (Raynaud’s phenomenon and chilblain-like lesions), permanent acrosyndromes (acrocyanosis), and transient acrosyndromes, in which their pathogeneses are associated with virus infections, Epstein-Barr virus, and, more recently, SARS-CoV-2, respectively.MethodsWe reported a case of BASCULE syndrome associated with postural orthostatic tachycardia syndrome (POTS) and provided a narrative review of case reports describing the BASCULE syndrome in children. Moreover, we presented the results of a prospective practice survey that we performed in the French medical community.ResultsA 14-years-old boy reported pruritic erythrocyanic lesions on the lower limbs, which occurred whenever he was in a standing position and fully resolved when he laid down. He reported asthenia and cramps. He presented a typical BASCULE syndrome associated with POTS confirmed by a tilt-test. Physical and vascular examinations were within the normal range. We identified 12 case reports, describing 21 pediatric cases since 2016. Most patients were adolescents between 12 and 19 years of age or were newborns. Furthermore, 20% of cases in the literature have presented POTS or orthostatic intolerance. Our survey among 95 French physicians confirmed that BASCULE syndrome is an underdiagnosed and under recognized disease in the general pediatric practice, at least in France. Among these physicians, 65% had already encountered patients with similar symptoms, but only 30% declared that they had knowledge of the BASCULE syndrome.ConclusionThe under-recognition of the clinical manifestations leads the patients to consult emergency rooms, with multiple unnecessary investigations performed. Therefore, we suggest that the diagnosis of BASCULE syndrome is based on clinical observations, without the need for laboratory tests, to avoid unnecessary health costs. We suggest physicians to perform a tilt-test when POTS is suspected.

show abstract

Section: Discussionmentioning

confidence: 99%

Case Report, Practices Survey and Literature Review of an Under-Recognized Pediatric Vascular Disorder: The BASCULE Syndrome

et al. 2022

View full text Add to dashboard Cite

show abstract

“…First reported in 2016, BASCULE syndrome is an uncommon vasomotor dermatosis that occurs most frequently in females aged 12-15 years. 1 It can be mistaken for urticaria, but the triad of clinical signs are differentiating and diagnostic. Bier anemic spots result from a poor venoarteriolar reflex in the dermal ascending arterioles, and the central urticaria-like lesions are secondary to mast cell degranulation induced by hypoxia.…”

mentioning

confidence: 99%

“…2 The eruption is triggered by standing but resolves with lying down or walking. 1 Pruritus, pain and edema may be present and may warrant treatment. 1 The importance of diagnosing BASCULE syndrome lies in its association with a spectrum of orthostatic intolerance conditions, including syncope, orthostatic hypotension and POTS (a condition associated with mast cell activation and hypermobility spectrum disorders).…”

mentioning

confidence: 99%

“…1 Pruritus, pain and edema may be present and may warrant treatment. 1 The importance of diagnosing BASCULE syndrome lies in its association with a spectrum of orthostatic intolerance conditions, including syncope, orthostatic hypotension and POTS (a condition associated with mast cell activation and hypermobility spectrum disorders). 2,3 Less commonly, it is associated with changes detected by electrocardiography, including prolonged QT interval and first-degree atrioventricular block.…”

mentioning

confidence: 99%

“…2,3 Less commonly, it is associated with changes detected by electrocardiography, including prolonged QT interval and first-degree atrioventricular block. 1 A diagnosis of BASCULE syndrome should prompt assessment for orthostatic intolerance and electrocardiogram abnormalities. Involvement of a cardiologist and an allergist or immunologist may be considered.…”

mentioning

confidence: 99%

See 2 more Smart Citations