When alpha-gal hides: a case series of atypical presentations of alpha-gal syndrome in children

Abstract Background Alpha-gal syndrome is an IgE-mediated allergy resulting from sensitisation to galactose-alpha-1,3-galactose (alpha-gal). Although classically associated with delayed urticaria or anaphylaxis following red meat ingestion, it can present with diverse and atypical manifestations. We report four paediatric patients with rare and atypical manifestations of alpha-gal syndrome. Case presentations Case 1: An 18-month-old boy with recurrent respiratory symptoms was on treatment for allergic rhinitis and poorly controlled asthma for six months. His symptoms showed an inconsistent association with dairy consumption; however, there were no cutaneous manifestations. Case 2: An 11-year-old boy with recurrent abdominal pain and altered bowel habits for one year was managed as irritable bowel syndrome. His symptoms frequently followed ingestion of dairy products. Case 3: An 8-year-old boy had been experiencing brief episodes of urticaria for four months. He was treated as having chronic spontaneous urticaria with poor response to standard treatment. Case 4: An 18-year-old girl had 19 episodes of idiopathic anaphylaxis since the age of six years, with symptoms that were not consistently associated with an identifiable food trigger. All patients recalled a history of tick bites. Detailed history revealed delayed symptom onset and inconsistent reactions to alpha-gal-containing foods. The diagnosis of alpha-gal syndrome was confirmed in all four patients by skin prick testing and elevated specific IgE for alpha-gal. Elimination of the implicated food allergens led to complete resolution in all cases. Conclusions This case series illustrates the diverse and atypical clinical presentations of alpha-gal syndrome in children which can lead to delayed recognition and misdiagnosis. Increased clinician awareness is the key to early diagnosis, thus preventing recurrent episodes of symptoms and anaphylaxis and improve overall outcomes.