BASCULE Syndrome in Young Children: Two Cases With Temporally Associated Viral Infections and Diagnostic Challenges.

Abstract

Bier anaemic spots, cyanosis and urticaria-like eruption (BASCULE) syndrome is a rare vasomotor disorder reported predominantly in adolescents. It is characterized by a distinctive triad of Bier spots, cyanotic discolouration and urticarial lesions, typically triggered by orthostatic stress. We report two cases occurring in young children aged 16 months and 4 years, an age group in which the syndrome is rarely documented and in which the presentation is easily mistaken for vasculitis, drug eruption or sepsis. In both children, the eruption was preceded by a viral infection (Epstein-Barr virus and influenza A, respectively) and both children showed morphological variants that are not classically described: unilateral acral cyanosis with refusal to bear weight in the toddler and a generalized, purpuric-appearing febrile eruption in the preschool child. In both patients, the cutaneous findings cleared within minutes of recumbency, and neither child had syncope, presyncope or other features of orthostatic intolerance. The eruption resolved with supportive care, and there was no recurrence during follow-up. The temporal relationship with viral infection observed here is consistent with previous anecdotal reports but does not establish causality, which remains unproven. Recognizing BASCULE syndrome in young children may prevent unnecessary investigations and reduce patient and caregiver anxiety.