Refractory Noninfectious Anterior Uveitis Associated With Systemic Mastocytosis

A 10-year-old boy with biopsy-proven systemic mastocytosis presented with bilateral nongranulomatous anterior uveitis associated with high intraocular pressure. Anterior chamber paracentesis was performed for cytological evaluation, which demonstrated monocytes and macrophages but no evidence of mast cells. His uveitis was partially controlled with frequent topical steroid drops and weekly oral methotrexate therapy. The glaucoma was controlled with topical antiglaucoma medications in the right eye. Deep sclerectomy was required in the left eye to control the intraocular pressure. To the best of the authors' knowledge, this is the first report of such an association.