Bilateral Parry-Romberg syndrome associated with retinal vasculitis

PURPOSE. To describe an unusual case of bilateral progressive facial hemiatrophy (Parry-Romberg syndrome (PRS)) associated with retinal vasculitis. METHODS. In a 37-year-old man with bilateral PRS, retinal vasculitis of the right eye was evident on fundus examination and fluorescein angiography. Right temporalis muscle biopsy and needle electromyography of the masseter muscles were performed. The patient underwent immunosuppressive therapy and retinal laser photocoagulation. RESULTS. Biopsy specimens showed large fibrosis with focal lymphohistiocytic infiltration of the muscle fibers. Electromyographic findings are consistent with a primary muscle disease. Visual acuity improved from 20/25 to 20/20 in the right eye with a follow-up of one year. CONCLUSIONS. The evidence of retinal vasculitis and the histologic findings of facial changes observed in this PRS case could support the pathogenetic model of a chronic inflammatory process as a plausible explanation for progressive facial hemiatrophy.