Necrosis of the tongue in a patient with intestinal infarction

A. Patterson, FRCS,” C. Scully, MD, PhD, MDS, FDS, FFD, MK’Path,b N. Barnard, FKCS,’ M. J. Grifiths, MBBS, FDS,d J. W. Eveson, PhD, FDS, FRCPath,” and M. Novelli, MSc, MBBS! Bristol,

England

CENTRE FOR THE STUDY OF ORAL DISEASE, UNIVERSITY DEPARTMENT OF ORAL MEDICINE, SURGERY, AND PATHOLOGY, ERISTOL DENTAL HOSPITAL AND SCHOOL A patient with a rare combination of bilateral lingual necrosis giant cell arteritis, is described and the literature reviewed. (ORAL SURC ORAL MED ORAL PATHOL

and intestinal

infarction,

caused by

1992;74:582-6)

iant cell, or temporal, arteritis is a granulomatous arteritis, sometimes termed Horton’s disease,’ which involves medium and larger arteries, particularly

branches of the aortic arch, and is found mainly in older women2 It is closely related to polymyalgia

rheumatica. Apart from low-grade fever, anorexia, weight loss and generalized aches and pains, there are often manifestations in the head and neck including temporal pain and tenderness, headache, and neuro-ophthalmic features, particularly sudden blindness. Patients may also have jaw or masseteric pain, particularly during chewing,4T 5 or may have pain in the tongue.3, 6-8Lingual arteritis is common: one autopsy

study of five patients showed lingual arteritis in 9 of 10 arteries examined.’ Although the tongue has an excellent blood supply and usually survives, lingual necrosis has now been described in nearly 50 patients with giant cell arteritis (Table 13,7-53). The lingual

Anterior and bilateral lingual &hernia with necrosisstarting centrally. Large massof mucusis adherent betweenpalate and dorsumof tongue.

necrosisis almost invariably unilateral, and only very

CASE REPORT

rare cases of bilateral necrosis have been reported 12,13,15,35 Lip necrosis has also been reported in giant cell arteritis.54 We report a case of rare bilateral lingual necrosis

An 82-year-old white womanwasadmitted to hospital becauseof acute abdominalpain and fever that proved to be relatedto infarction of a segmentof smallintestine.Two weekslater her conditiongenerallydeterioratedanda black appearanceand pain developedin her tongue.Apart from

in a patient who also had giant cell arteritis with intestinal lesions, and review the literature. aRegistrar. bProfessor. %enior Registrar. dConsultant. eReader. fSenior House Officer. 7113138357

582

mild congestive heart failure, osteoarthrosis, and a history of renal calculi, her medicalhistory wasnoncontributory, and specifically she had no myalgia, temporal pain or headache, or deterioration of vision to suggest giant cell arteritis. A dermatologic opinion suggested a diagnosis of “black hairy tongue”; an oral medicine opinion was sought.

Oral examinationrevealeda slightly swollentonguethat anteriorly appeared blackened and gangrenous (Fig. l), was restricted in movement, and was painful to palpation. The

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Tongue necrosis in patients with intestinal

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Fig. 2. Lingual artery showing intimal thickening with inflammatory infiltration of media and intima and associated multinucleated giant cells. (Hematoxylin-eosin stain; original magnification, ~60.)

Fig. 4. Temporal artery showing intimal proliferation almost obliterating lumen and scattered giant cells. (Hematoxylin-eosin stain; original magnification, X60.)

DISCWSSION

This patient with giant cell arteritis had manifestations in the head and neck, and gastrointestinal tract, but few positive findings from investigations. Giant cell arteritis classically presents as headache but atypical presentations often result in a delay in diagnosis for weeks or months. Manifestations fall into four major groups: cranial features; polymyalgia rheumatica; constitutional features such as fever, fatigue, anorexia, and weight loss; and pulmonary

Fig. 3. Lingual artery showing fragmentation of elastic lamina and multinucleated giant cells. (Hematoxylin-eosin stain; original magnification, X 150.)

provisional clinical diagnosis was of lingual infarction as part of a generalized vasculitis such as giant cell arteritis. Investigations included estimation of the erythrocyte sedimentation rate (ESR.) (raised); biopsy of the tongue, which showed necrotic tissue infected with gram-negative bacilli but. no evidence of arteritis; and a temporal artery biopsy (no abnormality dyetected). With the patient under general anesthesia, the necrotic lingual tissue was resected back to vital lingual tissues; giant cell arteritis was evident (Figs. 2 and 3). However, during the next 3 weeks severe headaches, which failed tlo respond to systemic corticosteroids, developed and eventually required diamorphine for control, and she finally died. Autopsy confirmed a disseminated vasculitis indicative of giant cell arteritis, with intestinal perforation, and lesions affecting the temporal artery (Fig. 4) despite the previous negative findings on temporal artery biopsy.

symptoms such as dyspnea and cough.55 The presentation may therefore initially suggest conditions other than an arteritis.s6 Early diagnosis of giant cell arteritis is important particularly because sudden blindness is a complication.57 Physical

examination

shows a tender, thick-

ened, and nodular temporal artery in 70% of patients, and in some patients there is a loss of arterial pulsation. Ischemic ulcers of the scalp, or hair loss, may be present. Bruits may be audible over the carotid and subclavian arteries. Jaw claudication

or lingual pain,

ulceration, or necrosis may be seen in isolation or with other features (Table I). Giant cell arteritis is rarely associated with overt gastrointestinal complications, although Crohn’s disease has been reportedly associated58 and a few patients have had intestinal infarction5gs 6o or perforation or fistula formation.61, 12 The main differential diagnoses of giant cell arteritis affecting the head and neck are other causes of headache and orofacial pain, and a miscellany of less common conditions. Thus it is sometimes important to exclude migraine, migrainous neuralgia, paroxysmal trigeminal neuralgia, and atypical facial pain. Poly-

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Table 1. Lingual &hernia and necrosis in giant cell arteritis Patient Comments Author

Year

Age (~4

Gender

Grahame et al. Howard and Cremin McGill Pitt et al. Missen Brearley and McDonald Reed and In&

1968 1959 1961 1961 1961 1961 1965

15 74 77 78 NR 82 12

F F F F NR F F

Davis and Davis Henderson Wolpaw and Brottem Higgins Arnung and Nielsom Hicks and Lee Allen Sofferman

1966 1961 1973 1973 1979 1980 1980 1980

Dare

1981

74 78 68 90 69 73 66 68 68 74 82

F M F F F F F F F M M

69 89 12 77 58 66 78 79 NR 74 17 68 34 76 80 80 79 81 81 76 54 77 75 78 77 12 80 70 84 70

M F F F M M F F NR F F F M F M F F M F M F M M F M M F F F M

et al.

1

Browne

1982

Storm and Jorgensen Pedersen and Jepsen Roseman and Granite Barfoed and Bretlau Jenkins Bowdler and Knight Siemssen et al. Christensen Jadoul et al. Honberg Rubin et al. Horing and Wienbeck Schweitzer and DeJong Bergman Kinmont and McCallum Freedman and Hooley Harris Bour et al. Schulz Feraut Samson et al. Reboul et al. Beylot et al. Dufetelle et al. Doumith et al. Remky et al. Renard et al. Hujic and Piranic

1983 1983 1984 1984 1985 1985 1985 1986 L987 1989 1990 1990 1949 ?959 1964. 1967 1968 1969 1971 1971 1974 1975 1975 1976 1981 1981 1982 1982

arteritis nodosa, malignancy, and other causesmust also be excluded. The most helpful investigations are estimation of the ESR and temporal artery biopsy.s7,63-65The ESR is typically raised, but up to 20% of patients do not

and additional features

Lingual ischemia; no necrosis Bilateral lingual necrosis Oculomotor palsy Bilateral lingual necrosis Scalp necrosis; bilateral lingual necrosis Aching pains in temporal areas radiating jaws

Tongue Necrosis Bilateral

cyanosis

after

to

ergotamine

of floor of mouth lingual necrosis

and scalp

Sudden onset bilateral lingual pain and swelling; previous jaw claudication and transient blindness

Sudden blindness Facial pain Ergotamine implicated Severe headache Facial

pain and headache

Painful tongue mass; no necrosis Scalp necrosis Blindness in one eye Reduced

vision

Aortic aneurysm Blindness; scalp necrosis Scalp necrosis Blindness; scalp necrosis Temporary blind; scalp necrosis Blindness; scalp necrosis

have a raised ESR.64 Arteritis affects the arteries in a patchy manner,‘j5and therefore biopsy may be negative as in our case. Temporal artery biopsy is positive in up to only 50% in some studies. If the first biopsy findings are negative, a second is indicated.5i

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Tongue necrosis in patients with intestinal

Nevertheless, the diagnosis may have to be made on the basis of clinical characteristics alone, and nn view of the risk of blindness, therapy may need to be instituted even in the absence of positive findings from investigations. Systemic corticosteroids are usually remarkably effective, typically ameliorating the symptoms and lowering the ESR within a few days. Large dosages of prednisone (40 to 150mg/day) for up to 4 weeks are best followed by a maintenance dosage (monitored by ESR) for up to 2 years after presentation. U-Jnfortunately, intestinal complications may appear after the successful treatment of head and neck manifestations with corticosteroids62 and may, as here, prove lethal. REFERENCES 1. Horton BT. Headaches and intermittent claudication of the jaw in temporal arteritis. Headache 1962;2:29-40. 2. Klein RG, Hunder GG, Stanson AW, Sheps SG. Large artery involvement in giant cell (temporal) arteritis. Ann Intern Med 1975;83:806-12. 3. Friedlander AH, Runyan C. Polymyalgia rheumatica and temporal arteritis. O~RAL SURG ORAL MED ORAL PATHOL 1990;69:317-21. 4. Jones JG, Hazleman BL. Giant cell arteritis presenting to the dentist: a need for urgent diagnosis. J Dent 1983;11:356-60. 5. Lamey PJ, Taylor JA, Devine J. Giant cell arteritis: a forgotten diagnosis? Br Dent J 1988;164:4.8-50. 6. Henderson AH. Tongue pain and giant cell arteritis. Br Med J 1967;4:337. 7. Grahame R, Bluestone R, Holt PJ. Recurrent blanching of the tongue due to giant cell arteritis. Ann Intern Med 1968;69: 781-2. 8. Rubin MM, Gatta CA, Cozzi GM, Rogerson KC. Painful tongue mass. J Oral Maxillofac Surg 1990;48:728-31. 9. Missen GA. Gangreneof the tongue. Br Med J 1961;1:1393-4. 10. Schweitzer PMJ, DeJong JGY. Een geval van arteriitis temporalis met oogafwijkingen aan de slijmvliezen. Ned Tijdschr Geneeskd 1949;93:2536. 11. Bergman JJ. Ischemic necrosis of the tongue. Q Bull Northwestern Univ Med School 1959;33:38-40. 12. Howard S, Cremin MD. Acute parenchymatous glossitis with gangrene of the tongue. Lancet 1959;2:4 lo- 1. 13. Brearley BF, MacDonald JG. Temporal arteritis resulting in infected gangrene of the tongue. Br Med J 1961;1:1151-2. 14. McGillRJ.Gangreneofthetongue.BrMed J 1961;1:1610-1. 15. Pitt P, Wallace I, MacGregor GA. Gangrene of the tongue. Br Med J 1961;1:1394. 16. Kinmont PDC, McCallum DI. Skin manifestations of giantcell arteritis. Br J Dermatol 1964;76:299-308. 17. Haye C, Rousselie F, Mondon J. Bilateral blindness due to Horton’s disease associated with extensive scalp and tongue necrosis. Bull Mem Sot Franc Ophtholmol 1967;80:617-8. 18. Reed C, Inglis MJ. Acute massive gangrene of the tongue. Br Med J 1965;2:575-6. 19. Davis AE, Davis TP. (Gangrene of the tongue caused by temporal arteritis. Med J Aust 1966;2:459. 20. Freedman GL, Hooley JR. Ischemic necrosis of the tongue. ORALSURGORALMED ORALPATHQL 1967;24:821-4. 21. Harris M. Dissecting aneurysm of the aorta due to giant-cell arteritis. Br Heart J 1968;30:840-4. 22. Bour H, Renoux M, Guy-Grand B, Tutin M, Costill J, Escande C. Maladie de Horton. Presse Med 1969:77:233-6. 23. Schulz P. Zungennekrose bei Altersarteriitis (Arteriitis temporalis). Zahnaerztliche Welt 1971;80:932.

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24. Feraut D. Les gangrenes au tours de la maladie de Horton [These]. University of Bordeaux: 1971:115. 25. Allen P. Giant cell arteritis presenting with necrosis of the tongue: a case report. Br J Oral Surg 1980;18:162-5. 26. Higgins AIT. Ulcerative and sequestrating glossitis in giant cell arteritis. Schweiz Med Wochenschr 1973;103:1463-5. 27. Wolpaw JR, Brottem JL. Tongue necrosis attributed to temporal arteritis. JAMA 1973;225:514. 28. Samson M, Augustin P, Verdure L, Fondimare, A. Un cas de maladie de Horton avec necrose etendue de l’extremite cephalique. Rev Oto Neuro Ophthalmol 1974;46:183-8. 29. Reboul M, Boissonnas A, Cachin M. Necrose du cuir chevelu et de la langue revelant une maladie de Horton. Nouv Presse Med 1975;4:1917. 30. Beylot C, Loiseau P, Beylot J. Gangrenes au tours de la maladie de Horton. Nouv Presse Med 1975;4:2948. 31. Dufetelle JP, Tapie B. Necrose linguale au tours d’une maladie de Horton. Rev Stomatol 1976;77:1013-9. 32. Arnung K, Nielsom IL. Temporal arteritis and gangrene of the tongue. Acta Med Stand 1979;206:239-40. 33. Hicks K, Lee FI. Necrosis of the tongue secondary to cranial arteritis. Br J Oral Surg 1980;18:166-9. 34. Doumith R, Gaudin P, Bertrand J-C, Goudal J-Y, Ragot J-P. Necrose linguale reveletrice dune maladie de Horton. Rev Stomatol Chir Maxillofac 1981;82:66-9. 35. Dare B, Byrne E, Robertson A. Acute lingual ischaemia complicating temporal arteritis. Med J Aust 1981;1:534. 36. Remky A, Pellkofer M, Spitzer H. Zungennekrose und beidseitiger Optikusbefall bei Arteritis cranialis. Munchener Med Wochenschr 1981;123:225-6. RA. Cranial arteritis in otolaryngology. Ann Otol 37. Sofferman Rhino1 Laryngol 1980;89:215-9. 38. Browne W. Oral necrosis accompanying giant cell arteritis. J Oral Maxillofac Surg 1982;40:7, 450-6. 39. Renard JP, Chavanne D, Jacquesson M, Groussin P. Necrose linguale au tours de la maladie de Horton. Sem Hop Paris 1982;58:51-2. 40. Hujic H, Piranic H. Tongue necrosis in Horton disease. Stomatol Vejsnik 1982;11:13. in giant cell ar41. Pedersen AT, Jepsen FL. Lingual infarction teritis. J Laryngol Otol 1983;97:479-83. 42. Storm TL, Jorgensen F. Lingual infarction and sudden blindness due to giant cell arteritis. Acta Med Stand 1983;214:85-6. 43. Symanzik J. Riesenzellarteriitis als Ursache fur eine Zungennekrose. HNO 1984;32:115-8. 44. Roseman BB, Granite E. Massive tongue necrosis secondary to temporal arteritis. J Oral Maxillofac Surg 1984;42:682-4. 45. Jenkins PR. Lingual infarction on cranial arteritis. Dent Update 1985;12:303-5. 46. Christensen L. Ulceration and necrosis of the tongue due to giant cell arteritis. Acta Med Stand 1986;220:379-80. 47. Wolpaw JR, Brotten JL, Martin HL. Tongue necrosis attributed to ergotamine in temporal arteritis. JAMA 1973;225: 514-5. 48. Honberg PZ. Giant cell arteritis and gangrene of the tongue. Ugeskr Laeger 1989;151:637-8. 49. Jadoul M, Lambert M, Huaux JP. Lingual necrosis as the presenting feature of temporal arteritis. Acta Clin Belg 1987;42:473-4. 50. Barfoed CP, Bretlau P. Tongue necrosis in temporal arteritis. Acta Otolaryngol 1984;98:380-4. 51. Bowdler DA, Knight JR. Lingual claudication and necrosis as a complication of giant cell arteritis. J Laryngol Otol 1985; 99:417-20. 52. Siemssen SJ. On the occurrence of necrotising lesions in arteritis temporalis: review of the literature with note on the potential risk of a biopsy. Br J Plast Surg 1987;40:73-82. mit Kopfhaut und 53. Horing E, Wienbeck M. Arteritis temporalis Zungennekrose sowie mit Stammhirninsult. Med Klin 1990; 85:159-60. 54. Pogrel MA. Necrosis of the upper lip from giant cell arteritis. J Oral Maxillofac Surg 1985;43:300-2.

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November 55. Desmet GD, Knockaert DC, Bobbaers HJ. Temporal arteritis: the silent presentation and delay in diagnosis. J Intern Med 1990;227:237-44. 56. Healy LA, Witske KR. Presentation of occult giant cell arteritis. Arthritis Rheum 1980;23:641-3. 57. Huston KA, Hunder GG, Lie JT, Kennedy RH, Elveback LR. Temporal arteritis: a 25-year epidemiologic, clinical and pathologic study. Ann Intern Med 1978;88:162-7. 58. Teja K, Crum CP, Friedman C. Giant cell arteritis and Crohn’s disease: an unreported association. Gastroenterology 1980;78: 796-802. 59. Stenwig JT. Intestinal gangrene due to giant cell arteritis: a report of a case. J Oslo-City Hosp 1976;26:49-55. 60. SrielevSR. Gardiner GW. Giant cell arteritis with small bowel inf&tion:‘a case report and review of the literature. Am J Gastroenterol 1980;73:157-61. 61. Smith JAE, O’Sullivan M, Gough J, Williams BD. Small intestinal perforation secondary to localized giant cell arteritis of the messenteric vessels. Br J Rheumatol 1988;27:236-8.

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62. Patsner B, Pitzele S, Mann WJ, Chalas E. Temporal arteriiis and intestinal perforation. N Y State J Med 1989;8:476-7. 63. Jonasson F, Cullen JF, Elton RA. Temporal arteritis: a ICyear epidemiological, clinical and prognostic study. Scot Med J 1979;24:11 l-7. 64. Ellis ME, Ralston S. The ESR in the diagnosis and management of the polymyalgia rheumatics/giant cell arteritis syndrome. Ann Rheum Dis 1983;42:168-70. 65. Klein RG, Campbell RJ, Hunder GG, Carney JA. Skip lesions in temporal arteritis. Mayo Clin Proc 1976;51:504-10. Reprint requests: Prof. C. Scully Centre for the Study of Oral Disease University Department of Oral Medicine, Pathology Bristol Dental Hospital and School Lower Maudlin Street Bristol, U.K. BSl 2LY

Surgery

and