Objective: Giant cell arteritis (GCA) often presents with devastating visual loss in the elderly, yet early diagnosis and treatment can prevent visual loss. The authors report two cases of GCA in which scalp necrosis, a rare finding not reported previously in the ophthalmic literature, played an important role in diagnosis. Design: Observational case reports and literature review. Participants: Two patients with GCA had scalp necrosis. Intervention: Intravenous steroid administration was performed. Main Outcome Measure: Vision and healing of scalp necrosis were measured. Results: One patient had scalp necrosis that was diagnosed incorrectly preceding visual loss. Another patient had visual loss in one eye diagnosed as nonarteritic anterior ischemic optic neuropathy. He had scalp necrosis develop 2 weeks later, leading to the correct diagnosis of GCA. There are 21 previously reported cases of scalp necrosis with GCA, none in the ophthalmic literature, most of which were undiagnosed until the onset of visual loss. In addition, a higher rate of visual loss is seen in GCA with scalp necrosis. Conclusions: Scalp necrosis is a valuable sign that frequently is misdiagnosed until visual loss occurs. In addition, it may indicate a more severe vasculitis. This finding should be added to the list of signs evaluated in patients for whom GCA is in the differential diagnosis.
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