Uveitis in Parry–Romberg syndrome: A case report and review of literature

Parry-Romberg syndrome (PRS) is a rare, acquired condition characterized by progressive atrophy of the skin and soft tissue of one side. A 35-year-old female with a 4-year history of recurrent uveitis and hypotony in the right eye, previously diagnosed with granulomatous anterior uveitis and pars planitis, was referred to our clinic. Despite treatment, she continued to develop recurrences of inflammation, with intraocular pressure dropping to 0 mmHg twice. She was managed with periocular injection triamcinolone acetonide with systemic anti-inflammatory treatment. A dermatology consultation was sought based on a general examination that revealed hyperpigmented lesions on the right side of the forehead and scalp, as well as alopecia patches. The dermatologist diagnosed her with PRS. She was managed with oral methotrexate and subsequently did not develop any further recurrence of inflammation or hypotony. A literature search revealed 23 cases of PRS with uveitis. Most cases reported an early onset of PRS preceding a uveitis episode. The most common subtypes of uveitis were retinal vasculitis and Coats'-like response that were associated with inflammation in the anterior chamber and vitreous. PRS can be a rare cause of uveitis but can be often overlooked by clinicians. Such patients should be monitored closely, as they may develop ocular hypotony over time, but can be managed with aggressive treatment.