Chalky Pallid Edema

The patient was hospitalized and received 1 g/day of intravenous methylprednisolone for three days followed by oral prednisone at 80 mg/day. A right temporal artery biopsy (TAB) showed intimal calcific deposits but was without granulomatous inflammation or vasculitis. Three weeks after initial presentation, the patient noted new headaches despite steroid usage. Visual acuity dropped to hand motions in the right eye and visual fields showed severe diffuse vision loss. Visual acuity and fields were full in the left eye. Despite the patient's relatively young age and negative TAB, GCA was still considered a possibility given the prominent jaw claudication. Prednisone was tapered to 40 mg/day over months. Seven months after presentation, prednisone had been increased back up to 40 mg a day due to recurrent headaches and jaw claudication. Despite this, the patient developed dyschromatopsia and superior disturbance in her left eye. Visual acuity in the left eye remained 20/20, but visual fields showed a new superior altitudinal visual field defect. Fundus examination showed no disc edema or pallor of the left eye, and a left posterior ischemic optic neuropathy (PION) was diagnosed. The patient was hospitalized for further intravenous methylprednisolone. The patient also noted a new dark lesion over the left inguinal region. A biopsy of the skin lesion was consistent with calciphylaxis. In conjunction with nephrology, the patient was treated with sodium thiosulphate and the steroids were quickly tapered down. With the clinical context of calciphylaxis seen in the skin lesions, the TAB was reviewed with pathology who reinterpreted the calcific deposits of the temporal artery as consistent with calciphylaxis. A diagnosis of AION, PION and jaw claudication, all due to calciphylaxis of branches of the common carotid artery was made.