Abstract

A 15-year-old girl presented to Ophthalmology with persistent blepharoptosis, ectropion, and multiple nontender nodules involving her right upper eyelid. The patient had a history of similar large lesions of her right upper and lower eyelids that had been treated with excisions, systemic antibiotics, intralesional dexamethasone, and intralesional 5-fluorouracil. There was clinical concern for recurrent chalazion, and the patient underwent another excision. The specimen was relatively large for an eyelid excision, measuring 1.4 x 1.4 x 0.9 cm. Histological examination demonstrated multifocal folliculitis; some follicles were ruptured and associated with suppurative and granulomatous inflammation. Gram stain revealed gram positive cocci. These findings deviated from the more typical features seen in chalazia; namely, there was a conspicuous lack of sterile lipogranulomas. Detailed chart review revealed that the patient had underlying hyperimmunoglobulin E syndrome, also known as Job syndrome, complicated by multiple methicillin-resistant Staphylococcus aureus infections. We present this case to draw attention to the fact that minor histological deviations in banal specimens often reflect significant underlying infectious or immune pathologies. In a busy practice, it is easy to fly by such deviations. However, giant chalazia can be the first manifestation of primary immunodeficiencies such as hyperimmunoglobulin E syndrome, and prompt diagnosis of such syndromes can be lifesaving for patients.