European Archives of Oto-Rhino-Laryngology

, Volume 265, Issue 8, pp 993–998

Spontaneous bilateral necrosis of the tongue: a manifestation of giant cell arteritis?


    • Otolaryngology, Head and Neck SurgeryTechnical University Munich
  • Achim Berthele
    • Department of NeurologyTechnical University Munich
  • Marc Burghartz
    • Otolaryngology, Head and Neck SurgeryTechnical University Munich
  • Jan Kiefer
    • Otolaryngology, Head and Neck SurgeryTechnical University Munich
Case Report

DOI: 10.1007/s00405-007-0556-x

Cite this article as:
Schurr, C., Berthele, A., Burghartz, M. et al. Eur Arch Otorhinolaryngol (2008) 265: 993. doi:10.1007/s00405-007-0556-x


Giant cell arteritis is a chronic vasculitis, which involves large- and medium-sized branches of the arteries originating from the aortic arch. This disease is a diagnostic challenge with a wide range of clinical symptoms and findings due to different affected vessels. Classic symptoms are temporally located headache, thickened temporal artery and jaw claudication. Furthermore, visual symptoms like diplopia or loss of vision can occur. The tongue has an excellent blood supply and ischemic ulceration due to giant cell vasculitis is usually unilateral and rarely described in literature. We present a patient with a spontaneous bilateral tongue necrosis and are convinced that this extraordinary case must be deemed to be a manifestation of giant cell arteritis, although it does not completly satisfy the usually used diagnostic criteria formulated by the American College of Rheumatology.


Giant cell arteritisTongue necrosisLingual infarctionTemporal arteritisVasculitis


Giant cell arteritis is a chronic vasculitis, which involves large- and medium-sized branches of the arteries originating from the aortic arch. Generally this granulomatous vessel inflammation affects middle-aged and older persons, in 65% of women. It frequently occurs together with the disease of polymyalgia rheumatica (40%), which is characterized by aching and stiffness in the proximal joints [3, 9, 14, 15, 23, 24, 2931].

Giant cell arteritis is characterized by the presence of a patchy inflammatory infiltrate, which involves the inner media and the internal elastic lamina of the affected vessels with necrosis of smooth muscle. Commonly, a thrombus formation occurs in the affected vessels, which may be followed by obliteration of the lumen or recanalization [7]. Due to the course or distribution of the different affected arteries, the range of clinical symptoms and findings is wide.

Arteritis of the facial artery may cause jaw claudication in nearly 50% of the patients [1, 15, 23, 27]. Affection of the temporal artery leads to headache located in the temporal area and a thickened, pulseless vessel [1, 15, 17, 23, 27, 30, 31, 33]; in exceptional cases partial scalp necrosis may occur [8, 17, 34]. Ocular manifestations, including diplopia and loss of vision [15, 17, 23, 27, 2931, 33], may be due to arteritis of the ophthalmic artery, the posterior ciliary artery and the central retinal artery [1]. Rare manifestations are dysphagia in case of involvement of the ascending pharyngeal artery and sudden hearing loss with vertigo due to inflammation of the cochlear artery [1]. Ischemic ulceration or necrosis of the tongue due to giant cell vasculitis is extremely rare, because of its excellent blood supply. Furthermore, lingual infarction usually can be found unilaterally [17, 18, 20, 22, 38].

Generally, in case of tongue necrosis, several differential diagnoses are to be considered. We present a patient with a spontaneous bilateral tongue necrosis and are convinced that this manifestation originated from giant cell arteritis, even if the diagnostic criteria formulated by the American College of Rheumatology are not completely satisfied in this case (Fig. 1).
Fig. 1

Criteria formulated in 1990 by the American College of Rheumatology

Case presentation

Medical history

A 66-year-old woman with progredient neurologic symptoms was admitted to a hospital for the evaluation of a possible stroke. The patient had slurred, slow speech and a worsening dysphagia since 2 weeks. Her past medical history included among hypertension, a shaking palsy (M. Parkinson). She had no history of headache or fever. An outwardly performed cranial computed tomography was unremarkable. A grayish-black discoloration of the tongue attracted attention of the assigning hospital.

General constitution

The temperature was 36.8°C, the blood pressure 120/70 mmHg measured on the left arm. All peripheral pulses could be palpated. Examination of the heart revealed no abnormalities; an electrocardiogram showed a normal sinus rhythm at a rate of 76 beats per minute. Medical examination excluded significant coronary or pulmonary disease.

Neurologic examination

The patient was awake and well oriented andher memory was intact. She showed mild lethargy without any cognitive deficit. Motor function was decelerated with rigor. The cranial nerve functions were normal, except for a vertical gaze palsy. The sensations of a light touch, vibration and joint position were intact. Finger-to-nose and heel–knee–shin maneuvers showed slowed, but bilateral, correct coordination. The deep-tendon reflexes were equal and active bilaterally and ankle jerk was absent. Furthermore, a festinating gait and gait unsteadiness were detected. The rest of the neurologic exploration showed normal findings.

Ear–nose–throat investigation

Oral examination showed a slightly swollen tongue with a greyish-purple color of the anterior two-thirds. The tongue was movable in all directions and painful to palpation, while no suspicious ulceration or resistance could be determined. The bilateral duskiness of the tongue was sharply demarcated to the posterior third and base of the tongue, which showed normal smooth mucosa (Fig. 2). The elevation of the soft palate during phonation was symmetrical, and laryngeal examination showed a good bilateral glottic movement. Beyond this, enlarged lymphomas in the submandibular region could be detected. The remaining ENT investigations were without any remarkable findings. Temporal arterial pulsation was markedly alleviated. All parietal branches were not thickened, tender or erythematous. The patient had not noticed mandibular claudication at any time.
Fig. 2

Bilateral tongue discoloration and necrosis

Ophthalmologic examination

Ophthalmologic examination demonstrated a decreased vision on both eyes, without any visual field defect.

Laboratory findings

The white blood cell count was initially 10.41 G/l leukocytes and the C-reactive protein level was increased to 23.9 mg/dl. A remarkable sign of inflammation was the elevated erythrocyte sedimentation rate of 120 mm in the first hour. Further laboratory abnormalities were alterations in liver function (GOT, 64 U/l; GPT, 48 U/l; gamma-GT, 52 U/l) and a mild anemia (hemoglobin 11.3 g/dl, hematocrit 33.8%, erythrocytes 3.9 T/l). In addition, the clinical chemistry tests showed an increased creatininase raised to 621 U/l as an expression of muscle damage. A myocardial infarction was excluded by normal values of CK-MB and troponin T. All other laboratory findings were within normal limits, especially markers for an increased cardiovascular risk such as cholesterol, triglycerides or blood sugar levels.

Radiologic findings

The cervical ultrasonographic examination showed few submandibular lymphomas, but homogeneous salivary and thyroid glands. Thoracic radiography was unsuspicious.

Course of disease and further investigations

A prophylactic therapy with heparin was started to prevent any thromboembolic disease. Furthermore, an antibiotic medication was given to avoid local infection of the tongue. At this point of time, tumor, hematoma, embolism, abscess or a vasculitis disorder as reason for the tongue discoloration were considered.

A computed tomography angiographic study demonstrated a 3 cm long stenosis of 50% of the left common carotid artery with diffuse atherosclerotic changes. Both internal carotid arteries had patent vascular lumens, but the vertebral arteries presented with small calibers. The external carotid arteries, their branches and the tongue were artefactually distorted and could not be readily visualized, due to dental amalgam, but narrowed vessels and a necrosis of the tongue were suspected.

Therefore, a magnetic resonance imaging (MRI) of the head and neck and a diffusion-weighted magnetic resonance angiographic study were performed. These studies confirmed bilateral diffusely narrowed external carotid arteries and heavy stenoses starting near the bifurcation (Fig. 4), conformable with a vasculitis disorder. The lingual and the facial arteries could not be detected, possibly as a consequence of inadequate resolution or severe stenosis. Additionally, the imaging showed vessel irregularities of the vertebral arteries. Both common and internal carotids as well as all intracerebral arteries were not affected and were well perfused. No atherosclerotic changes could be seen. The intracranial findings were unsuspicious, without any cerebral ischemia. The MRI visualized a bilateral tongue necrosis as a hypointensive signal alteration (Fig. 5a, b).

Bilateral stenoses of external carotid and vertebral arteries and consecutive reduced blood flow were confirmed by carotid Doppler studies. The Doppler ultrasonography of both temporal arteries showed no halo or thickening of the vessel wall.

A malignant tumor, which may result in necrosis, was excluded by a tongue biopsy. The microscopic examination showed ischemic tongue infarction, but no tumor or signs of malignancy were found. With regard to the clinical, laboratory and radiological findings, we had, at this point, a strong suspicion of large-vessel vasculitis, including disease of the vertebral and external carotid arteries.

In addition to heparin and antibiotics, the patient was treated with intravenous steroids (500 mg prednisone) for 3 days, then with a reduced dose of 100 mg. After estimation of MRI and Doppler ultrasonography, a biopsy of the left temporal artery was performed under local anesthesia for histologic findings. The biopsy did not show a classic histologic picture of giant cell arteritis with granulomatous vessel inflammation. Merely, a moderate fibrous thickening of the intima was seen.

Meanwhile, the tip of the tongue appeared blackened, gangrenous and was restricted in movement. The anterior two-thirds were completely necrotic, with bilateral demarcations. However, the patient responded efficiently to the treatment, especially with a marked reduction in erythrocyte sedimentation rate, which decreased to 30 mm/h. Also C-reactive protein level and white blood cell count lowered as markers for disease activity and inflammation. The necrosis did not proceed and the remaining tongue showed scarred wound healing (Fig. 3).
Fig. 3

Wound healing 2 weeks after therapy started


This case did not satisfy the diagnosic criteria for giant cell arteritis of the American College of Rheumatology (Fig. 1) completely. Merely, the patient’s age of disease onset and the elevated erythrocyte sedimentation rate, two of the formulated five criteria were found. But, mainly the excellent therapeutic response to prednisone and the exclusion of differential diagnosis made the diagnosis definite. We are convinced that the patient’s diagnosis in this reported case was a giant cell arteritis and the tongue necrosis a rare disease manifestation. The tongue has an excellent blood supply, so ischemic ulceration or necrosis due to giant cell vasculitis is rare and only described in 50 patients [1, 2, 13, 19, 23, 24, 27, 36]. Lingual infarction is usually unilateral [17, 18, 20, 22, 38] and only extremely rare cases of bilateral necrosis have been reported [2, 23, 27, 37].

Generally, when tongue necrosis appears, besides vascular disorders, a variety of other causes have to be ruled out, such as malignant tumors affecting the tongue, e. g., carcinoma, lymphoma and sarcoma [2, 18]. Furthermore, embolism of the lingual artery, tongue infections or abscesses [2, 18] have to be considered as differential diagnoses as well as a previous history of head and neck radiotherapy or chemotherapy [17]. Therefore, exact anamnesis and diagnostic investigations are mandatory.

In the presented case, the performed angiographic magnetic resonance imaging (MRI) and computed tomography studies without contrast agent showed heavily narrowed external carotid arteries with diffuse wall changes, conformable with a vasculitis disorder, but well-perfused common and internal carotids without atherosclerotic changes (Fig. 4). A bilateral embolism of external carotid arteries seemed to be improbable. Except for the bilateral tongue necrosis (Fig. 5a, b), no tumor or abscess could be detected. Additionally, the microscopic examination of the tongue biopsy showed necrosis without signs of malignancy or inflammation.
Fig. 4

Angiography of the supraaortic arteries
Fig. 5

a Radiologic finding of tongue necrosis. b Radiologic finding of tongue necrosis

To ensure the diagnosis of giant cell arteritis, a further temporal biopsy was performed, but histology did not show the classic picture. Usually, artery walls are thickened by infiltration of lymphocytes, plasmacells and mononuclear histiocytes, together with 10–15% of multinucleated giant cells located in the junction between the intima and the media [1, 29]. We found a moderate fibrous thickening of the intima, without any cell infiltration. However, negative biopsy findings for the temporal artery cannot exclude giant cell arteritis [3], because the pathological lesion is typically segmental and localized only in fragments of the artery wall and is referred to as “skip-lesions” [9, 14, 27]. The disease is characterized by large segments of arteries presenting no lesion, while other segments may show signs of inflammation as evidence of the disease [3, 4, 7, 29]. So, temporal artery biopsy is only positive in 40–61% of the patients [15, 23] and the diagnosis in these cases is made based on clinical features [9].

Our patient showed highly suggestive laboratory abnormalities for giant cell arteritis. At hospitalization, we measured an increased C-reactive protein level, erythrocyte sedimentation rate (120 mm in the first hour), a moderate anemia and mildly abnormal liver-function tests, which are the typical laboratory findings of a large vessel disease [15, 17, 18, 24, 27, 29, 31]. In 89% of the patients with giant cell arteritis, an erythrocyte sedimentation rate greater than 50 mm/h can be registrated [15, 24, 35] and sometimes rates above 100 mm/h can be observed. Ronthal [28] pointed out that an erythrocyte sedimentation rate above 47 mm/h was 92% sensitive for giant cell arteritis. An embolism of the lingual artery without any tongue infection would not increase inflammation laboratory parameters.

Glucocorticosteroids are the cornerstone of treatment of giant cell arteritis, a dose of at least 40–60 mg of prednisone is mandatory for therapy. We performed a high-dose corticosteroid treatment with intravenous application of 500 mg prednisone for 3 days, followed by a reduced dose of 100 mg.

Generally, the response to corticosteroids is rapid, but a treatment course of 1–2 years is often needed. If the corticoid doses are reduced too quickly, symptoms can recur [25].

The clinical symptoms, the C-reactive protein value and the erythrocyte sedimentation rate are the most useful markers to monitor the therapy of giant cell arteritis [7, 29]. The presented patient responded efficiently to the corticoid treatment, with a reduction of these parameters and the tongue necrosis did not proceed (Fig. 3). This could be valued as a further indicator for the existence of the giant cell arteritis [10].

Almost every vascular pathway can be affected by giant cell arteritis and up to 40% of patients do not present with classic features [2]. Classic symptoms are temporally located headache in 60–90% of the patients [1, 15, 17, 23, 27, 30, 31, 33]. Pulseless, thickened and nodular temporal arteries are found in more than 50% of patients [15, 23, 29, 31]. Nearly half of the patients suffer from jaw claudication, which results from ischemia of the muscles of mastication [15, 23, 27, 35]. Visual symptoms, including diplopia and loss of vision, caused by an ischemic optic neuropathy develop in up to 20% [15, 17, 23, 27, 29, 31, 33]. Further classical findings are low-grade fever or weight loss in 70% [15, 23, 29, 30].

In approximately 10–15% of the patients, involvement of the subclavian and axillary arteries results in claudication of the upper extremity [15]. Atypical manifestations include dry cough, sore throat and tongue pain in 10% [15]. Head and neck complications including ischemic ulcers of the scalp, dysphagia, tongue necrosis, facial nerve palsy, vertigo or hearing loss are reported as individual cases [8, 17, 23, 27].

We are convinced, that this extraordinary case of bilateral tongue necrosis is a rare manifestation of giant cell arteritis. Two of the three postulated American College of Rheumatology criteria (Fig. 1) are satisfied, namely the patient’s age and increased erythrocyte sedimentation rate, which is an important diagnostic finding [18, 27, 29]. On the one hand the performed temporal artery biopsy was negative, but biopsy is not necessary for a definitive diagnosis and negative in a high percentage of cases (50% in some studies) [18]. Some authors believe that it can be omitted when giant cell arteritis is strongly suspected on clinical grounds [26]. Salvarani [29] states that the formulated criteria (Fig. 1) are not useful for making the diagnosis in individual cases, as they were initially designed for use in investigative studies to help distinguish giant cell arteritis from other types of vasculitis. Important to assure the diagnosis of giant cell arteritis is an improvement within 2 days of starting corticosteroid treatment [11], a course that could be observed in our case. Other causes for tongue necrosis (e.g., embolism and tumor) were screened out by tongue biopsy and radiologic investigation.


The bilateral tongue necrosis in the presented case must be deemed to be a rare manifestation of giant cell arteritis! In every case of atypical ischemic symptoms in combination with other unclear inflammatory symptoms, giant cell arteritis should be considered.

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© Springer-Verlag 2008