Adult Onset Asthma and Periocular Xanthogranuloma

Summary: Adult-asthma with periocular xanthogranuloma (AAPOX), an exceedingly rare disease, comprises one of four distinct subsets of adult orbital xanthogranulomatous diseases (AOX). AAPOX presents in the fourth and fifth decade of life with a male predominance. AAPOX was first described by Jakobiec et al. in 1993, where patients with periocular edema and eyelid plaques had a concomitant diagnosis of adult onset asthma. A 65 year old male patient with a past medical history of hypertension, gastroesophageal reflux disease and asthma initially presented with visual changes in 2020. He developed pruritus and periorbital swelling of his left eye associated with double vision. He was trialed on prednisone with resolution, but later had recurrence during the steroid taper . He was referred to ophthalmology where an orbital biopsy showed significant xanthomatous histiocytic proliferation. He was referred to rheumatology for further treatment and initiated on prednisone 60mg daily and methotrexate 15mg weekly with folic acid. One month after starting therapy, a repeat MRI showed complete resolution of periorbital swelling. He was able to successfully wean prednisone to 10 mg daily and maintained on methotrexate (MTX) 20 mg weekly. The plan is to continue to wean prednisone as able. We present a classic case of an extremely rare disease with successful treatment using prednisone and MTX as a steroid sparing agent. Additionally, this case helps emphasizes the importance of quick diagnosis and treatment to mitigate irreversible compromise to visual acuity.