Orbital angiolymphoid hyperplasia with eosinophilia in a pediatric patient: a case report

Abstract

A 7-year-old female presented to an outpatient eye clinic with a six-week history of left upper eyelid edema and erythema in the absence of trauma without compromise of visual acuity or extraocular muscle movements and was initially treated as a case of pre-septal cellulitis with antibiotic therapy. Failure of resolution with new onset nasal bleeding after two weeks prompted computed tomography (CT) and magnetic resonance imaging (MRI), showing an orbital mass with radiologic concern for lymphoma or rhabdomyosarcoma. Upon biopsy, the histopathological analysis diagnosed angiolymphoid hyperplasia with eosinophilia (ALHE), a rare condition that infrequently presents with ocular manifestations and more rarely presents in the pediatric population. Most therapeutic management for cutaneous and non-cutaneous ALHE have high rates of recurrence. This case was refractory to oral prednisone treatment but responded to a combination of methotrexate and adalimumab therapy with an 18.5% reduction in volume after three months.