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A variety of atypical manifestations in giant cell arteritis.

Giant cell arteritis (GCA) is a chronic granulomatous vasculitis of large and medium size vessels in the elderly. A new-onset headache is the most frequent symptom. An anterior ischemic optic neuropathy (AION) is one of the most common causes of permanent visual loss. There are four cases with unusual presentation of giant cell arteritis, scalp abscess, prolonged transient monocular visual loss (TMVL), bilateral central retinal artery occlusion (CRAO), and chronic ear pain. All patients had pathologically proven giant cell arteritis, and three of them progressed to blindness in the end. Scalp abscess is a rare sign in GCA. Delay in diagnosis because confusion of the abscess after scalp ischemia with other cutaneous lesions may result in death. TMVL is the forewarning symptom of AION or CRAO in GCA. Early recognition of TMVL is important to make early diagnosis of GCA to prevent blindness. Spontaneous ear pain is extremely rare, and reports have documented delay in diagnosis of GCA resulting in irreversible blindness.