Orbital Inflammatory Disease as a Presenting Symptom of Generalized Lipodystrophy in a Young Female

The authors report the first case of generalized lipodystrophy with orbital inflammatory symptoms. A 6-year-old female with hypothyroidism who developed hepatosplenomegaly, lymphadenopathy, and progressive loss of subcutaneous fat. Following flu vaccination, she developed orbital inflammatory symptoms. Imaging of the orbits demonstrated a paucity of retrobulbar fat and fat stranding. Systemic workup revealed insulin resistance and hepatosteatosis, consistent with generalized lipodystrophy. The authors discuss the typical history and examination findings in generalized lipodystrophy and review the etiology, treatment options, and outcomes.