Absence of esophageal eosinophilia in a toddler with severe eosinophilic esophagitis

Eosinophilic esophagitis (EoE) is a clinicopathologic disease characterised by esophageal dysfunction and eosinophil-predominant inflammation. Diagnosing EoE in children is particularly challenging as they tend to present with nonspecific symptoms and their mucosal specimens may have less than the threshold number of eosinophils. Reluctance to subject children to multiple endoscopic procedures and anesthesia often results in treatment with a proton pump inhibitor (PPI) first to evaluate clinical response. This approach presents a problem as a diagnosis of EoE may be missed. Here we present a case of a child with severe EoE whose initial biopsy did not show esophageal eosinophilia but progressed on to advance dis ease despite clinical response to PPI therapy.