Giant cell arteritis (GCA), one of the first causes of arteritis in the elderly, affects the vascular territory of the external carotid artery, especially in its superficial temporal branch1.
A 66-year-old patient was referred for a fever and rapidly progressive deterioration of his general state. The first symptoms appeared several days before, while the patient was visiting Thailand: fever and unusual bilateral headaches were followed by glossodynia and jaw claudication. Within about 2 h, tongue edema and jaw pain appeared. Intermittent binocular diplopia with transient amaurosis was also noted.
An erythematous and scabbed rash then developed on the patient’s scalp, featuring a necrosis area (Figure 1, and Supplementary Figures 1 and 2, available with the online version of this article). The tongue evolved toward cyanosis, further to necrosis (Figure 2, and Supplementary Figures 3–5, available with the online version of this article). The patient’s case history included bilateral shoulder arthralgia. C-reactive protein was 120 mg/l, and temporal artery biopsy confirmed the diagnosis of GCA. Treatment consisted of high-dose intravenous corticosteroids (methylprednisolone, 1.5 mg/kg/d for 3 d)2,3. Articular pain, headaches, and ophthalmologic symptoms drastically improved in < 48 h, while the scalp lesions responded at a slower rate. Emergency surgery for resection of the tongue necrotic tissue was performed. Because there was satisfactory primary healing, no further treatment was required.
The rich arterial supply network provided by the lingual artery usually prevents acute necrosis of the tongue body. However, even if they are rare, scalp4 and tongue5 necrosis are likely to simultaneously occur, as in our patient.
Better awareness of GCA initial signs and close collaboration among specialists could have allowed early diagnosis, thereby preventing such severe complications.
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Supplementary material accompanies the online version of this article.