Keratoconus Associated with Hyperimmunoglobulin E Syndrome

PURPOSETo report a case of keratoconus in a patient with hyper-IgE syndrome. METHODSA case report of a 28-year-old man with hyperimmunoglobulin E syndrome (HIES) who presented with chronic eczematous dermatitis of the eyelids and severe eye itching. RESULTSBest corrected visual acuity was 20/25 in the right eye and 20/30 in the left eye. There was a scissoring reflex in both eyes with retinoscopy. Biomicroscopy of the cornea was normal, but corneal topography showed bilateral keratoconus. CONCLUSIONSIt is difficult to establish a direct correlation between keratoconus and HIES due to the rarity of the latter disorder. It is, however, important to consider this association and obtain a corneal topography to rule out corneal ectasia in patients with HIES.