Case Report: Hyper IgE, but Not the Usual Suspects-Kimura Disease in an Adolescent Female

Abstract

Elevated immunoglobulin E (IgE) levels can be associated with infectious, allergic and inflammatory disorders, and rarely as a manifestation of an inborn error of immunity. Here we report the case of an adolescent female who presented with a gradually enlarging neck mass, lymphadenopathy, eosinophilia and highly elevated IgE levels. Laboratory and histopathologic evaluation revealed an unlikely diagnosis of Kimura Disease. We discuss the differential diagnosis of a neck mass with prominent eosinophils on histology, and review support for T-helper type 2 (Th2) cell activation and hyper-IgE in Kimura Disease.

Keywords: IgE; IgG4-related disease; Kimura Disease; T helper 2; angiolymphoid hyperplasia with eosinophilia; lymphocytic hypereosinophilic syndrome.