Severe Ophthalmic Involvement in Hyperimmunoglobulin E Syndrome; a Potentially Blinding Disease

Abstract Objective: To describe clinical and flow cytometry findings of an unusual case of hyper immunoglobulin E syndrome with severe ophthalmic involvements. Method: A Case report.Report: A 13-year-old boy was reported with infectious dermatitis, dental sinus with positive Actinomyces israelii culture, severe blepharitis, follicular conjunctivitis, and dysmorphic, vascularized cornea with no light perception visual acuity in both eyes. In immunophenotyping with the flow cytometry, a decrease in T-cell markers (CD2, CD3, CD4, CD5, CD7) was prominent. CD34 and CD117, hematopoietic stem cell, and circulating progenitor cell markers were negative.Conclusion: This is the first known reported case of flow cytometry findings, especially the lack of CD34+ and CD114+ cells in hyper immunoglobulin E patient with severe anterior and posterior segment involvement. Proper antimicrobial interventions are important in infectious flare-ups.