Tongue Necrosis Secondary to Giant Cell Arteritis

Giant cell arteritis is a form of vasculitis involving the medium- and large-sized arteries that chiefly affects older people. Clinical findings are headache, jaw claudication, fever, pain, and thickening of the temporal artery. The most feared complication is visual loss due to impairment of the ophthalmic artery and posterior ciliary arteries.

GCA occurs predominantly in females in a ratio of 1.4 to 3 women for every man and exclusively in patients aged 50 years and over.

A male, aged 85 years, arrived in the emergency department having suffered from frontotemporal headache associated with jaw pain over a period of 30 days. He reported that the pain had worsened during the last seven days and that pain had also developed on the floor of the mouth and chin. He also presented with a medical history of hypertension, cataracts, and osteoporosis. On admission, neurological examination and neuroimaging indicated no significant changes.

On the second day of hospitalization, the patient presented with increased lingual volume associated with intense pain and difficulty in eating. Inspection of the tongue revealed diffuse edema, whitish plaques, and a small aphthous ulcer. Treatment was initiated for oral candidiasis with fluconazole, and later Candida dubliniensis was isolated from a lingual swab. Within 24 hours, the lesion had changed colour, becoming greyish in a bilateral well-defined area, suggesting an ischemic lesion (Figure 1). Temporal pulses were absent and transient bilateral amaurosis appeared, thus confirming the presumptive diagnosis of GCA. Prednisone therapy was initiated with 1 mg/kg/day.

Clinical appearance of the tongue. (a) Tongue infarction at second day. (b) Initial auto-amputation of necrotic tongue at fifth day. (c) Tongue at 20th day presenting full epithelization.

Initial laboratory investigation indicated a complete blood count with mild leukocytosis, neutrophilia, thrombocytosis, and anemia (hemoglobin 11.7 g/dL, hematocrit 36%, 14,290 leukocytes/mm3 with 89% neutrophils, and 481,000 platelets/mm3). The erythrocyte sedimentation rate (ESR) of 120 mm/h and a C-reactive protein (CRP) of 17.2 mg/dl were consistent with an acute inflammatory process. Other laboratory tests were normal.

Doppler ultrasonography demonstrated that the temporal arteries were tortuous and presented diffuse intimal thickening with edema of the surrounding tissue (halo sign), and stenosis of around 75% of the light from the right temporal artery. A diagnosis of GCA was confirmed with a biopsy from the right temporal artery, which presented partial necrosis of the arterial wall with inflammatory infiltration mainly of mononuclear type (lymphocytes, histiocytes, plasma cells, and multinucleated giant cells) permeating the internal elastic lamina; calcification foci; and total lumen stenosis.

After glucocorticoid therapy was initiated, the patient presented a significant clinical and laboratory response. The area of the tongue that displayed the delimited necrotic lesion detached spontaneously within fourteen days and there was no progression of the ischemic region (Figure 1). After two weeks was initiated methotrexate 10 mg/week, and the patient was discharged clinically stable, and during his outpatient visits no new events or recurrences of GCA were reported. The prednisone dose was initially reduced by 10 mg every month until 20 mg/day and then reduced slowly. After twelve months, with evidence of normal inflammatory activity, the glucocorticoid was completely withdrawn.

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