LIFELONG PROGRESSIVE RETINAL ATROPHIC LESIONS IN A PATIENT WITH PARTIAL ACQUIRED LIPODYSTROPHY (BARRAQUER-SIMONS SYNDROME).

PURPOSE: To report a case of lifelong progressive retinal atrophic lesions in a patient with partial acquired lipodystrophy, that is, Barraquer-Simons syndrome.

METHODS: Case report.

RESULTS: A 67-year-old female patient with Barraquer-Simons syndrome was referred for progressive visual loss. Barraquer-Simons syndrome is a rare acquired partial lipodystrophy characterized by a loss of subcutaneous fat in the upper half of the body. Fundus examination disclosed posterior atrophic lesions, particularly evident on autofluorescence images, and their progression as compared to a previous examination performed nine years earlier. Multimodal imaging confirmed the posterior atrophic lesions without any associated exudative signs and highlighted the extension of atrophic areas in the periphery.

CONCLUSION: We here report for the first time a documented progressive retinal atrophy associated with Barraquer-Simons syndrome. Drusen and neovascular complications have already been described in previous reports, but no long-term follow-up was available until the present case to observe such dramatic evolution of the retinal lesions.