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.