QJM Advance Access originally published online on September 11, 2008
QJM 2008 101(11):909-911; doi:10.1093/qjmed/hcn117
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Tongue involvement revealing sarcoidosis
Sir,Sarcoidosis is an immune-mediated condition affecting multiple organs, especially the lungs, lymph nodes, skin and eyes.1–4 Oral involvement, including tongue manifestations, is considered to be a rare complication of sarcoidosis; in fact, to the best of our knowledge, there have been only five cases of patients exhibiting sardoidosis-associated tongue impairment, reported in the English literature.5–8 We recently observed the interesting case of a patient who developed tongue involvement as the first manifestation of sarcoidosis.
A 25-year-old woman, with unremarkable previous medical history, presented with a painless ulceration of 5-month duration, involving her tongue. The patient also complained of both fatigue and a 3 kg weight loss, and night sweats. At admission, oral examination demonstrated an ulcerated lesion with a central yellowish base, involving the inferior part of her tongue (Figure 1); general physical examination was otherwise normal. Laboratory findings disclosed the following: erythrocyte sedimentation rate (ESR): 10 mm/h, C-reactive protein: 14 mg/l, haemoglobin: 13.4 g/dl, white blood cell count: 5.1 x 109/l, platelet count: 225 x 109/l. Other routine biochemical tests, including glycaemia, renal and liver tests, vitamin B12 and folic acid blood levels, as well as blood and urinary protein immunoelectrophoresis, were normal. Blood cultures, urinalysis, as well as bacterial (Treponema pallidum, Mycoplasma pneumoniae, Chlamydia pneumoniae, Brucella), viral (cytomegalovirus, Epstein–Barr virus, Herpes simplex virus, Varicella–zoster virus, hepatitis B and C, human immunodeficiency virus) and Toxoplasma gondii serologies were negative. Auto-antibody screening also yielded negative results for: rheumatoid factors, anti-nuclear and anti-neutrophil cytoplasmic antibodies, anti-endomysium and anti-transglutaminase antibodies; serum angiotensin-converting enzyme level was elevated: 77 IU/l (N < 68).
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An incisional biopsy of the tongue lesion was performed. Histological examination of tongue lesion specimens showed numerous non-caseating epithelioid granulomas. Cultures of the tongue biopsy specimens were negative for mycobacterium species and fungi; polymerase chain reaction for Mycobacterium tuberculosis complex further proved negative. Tuberculin skin test was negative; further microbiological studies (Ziehl–Neelsen stain and cultures) in sputum and urine products were also negative for acid-fast bacilli. Thoracic and abdominal computed tomography (CT)-scan showed bilateral hilar lymphadenopathy and hepatomegaly. Pulmonary function tests revealed a mild decrease of diffusing capacity for carbon monoxide (69%) without restrictive pattern. Other investigations, including accessory salivary gland histological examination, gastroscopy and colonoscopy, were normal. A definite diagnosis of tongue involvement related to sarcoidosis was made. Because of the associated systemic manifestations (weight loss, asthenia and liver involvement) related to sarcoidosis, steroid therapy, at an initial dose of 20 mg daily was initiated. The patient's condition improved rapidly with: regression of clinical symptoms and tongue lesion.
Parotid gland (parotiditis, Herefordt syndrome) and minor salivary gland involvement is common in sarcoidosis, occurring in as high as 6% and 19–58% of patients, respectively.5–8 On the other hand, other oral manifestations are more uncommon complications in patients.5–8 In a review of the Medline database (1966–2008), we found only 71 well-documented reported cases of oral involvement sarcoidosis, supported by histopathological diagnosis.5–8 Analysis of these cases of oral sarcoidosis revealed a slight female predominance (40 females, 27 males; sex ratio: 1.5/1); patients ranged in age from 5 to 72 years, with the highest prevalence between 30 and 40 years.5–8
Oral involvement may be solitary or part of a generalized sarcoidosis. Indeed, in the literature review, oral involvement usually appeared in patients with chronic multisystem sarcoidosis, although it was the first manifestation of the disease in 35.2% of the cases.5–8 These data underscore that mouth examination should be systematically performed in patients with sarcoidosis. In our patient, tongue impairment revealed sarcoidosis. Our findings underline that when unexplained oral manifestations are noted, careful clinical examination should systematically be performed in order to detect underlying sarcoidosis. Oral clinical locations have been found to be variable. In our literature review, the jaw bones were the more common manifestations, occurring in 29.6% of patients; clinical signs were mainly due to lytic and permeatic lesions in the bone and included loose teeth.5–8 Other oral locations have further been described in patients with sarcoidosis, involving the buccal mucosa (18.3%; n = 13), the gingiva (16.9%; n = 12; gingivitis, gingival hyperplasia), the lips (8.5%), the floor of the mouth (8.5%; n = 6), the tongue (7%; n = 5), the palate (4.2%; n = 3), the submandibular glands (2.8%; n = 2); they were multiple in 4.2% of the patients (n = 3).5–8 Such sarcoidosis-related oral lesions may present as painless swelling, submucosal nodules or ulcerations;5–8 our case report confirms these data, as our patient exhibited a painless ulceration involving the tongue of 5-month duration.
Nevertheless, the definite diagnosis of sarcoidosis-related oral impairment can only be established after exclusion of other conditions.9–11 In our patient, a diagnosis of sarcoidosis-related tongue involvement could be made as: (i) the extensive search for other causes of tongue ulcerations was unsuccessful, including: infections (mainly tuberculosis, Herpes simplex virus, histoplasmosis and coccidiodomycosis), autoimmune diseases (especially Wegener's disease), Crohn's disease, as well as drugs-related ulcerations; and (ii) the resolution of all clinical and biochemical manifestations took place after initiation of therapy.
Therapy of sarcoidosis-related oral involvement remains controversial. In our literature search for therapy of oral sarcoidosis, we found data for 55 of these 71 patients with oral manifestations.5–8 We observed that 12 patients received no specific therapy; in 18 other patients, surgical excision and/or curettage was employed.5–8 Other procedures were used in the therapy of oral sarcoidosis, including steroids (n = 18) or other drugs (methotrexate, hydroxychloroquine or minocycline; n = 3) and oxygen (n = 1);5–8 previous authors have suggested that steroids should be considered in the group of patients who exhibit: (i) painful and progression lesions; and (ii) oral involvement associated with multivisceral and severe sarcoidosis.
Finally, our case report reinforces the possibility of an unusual presentation of sarcoidosis. Indeed, internists should be aware that tongue ulceration(s) may be related to sarcoidosis, resulting in earlier diagnosis and management. In such patients exhibiting unusual oral manifestations, diagnosis of sarcoidosis relies on compatible clinical signs, evidence of non-caseating granulomas and exclusion of underlying conditions, especially infections and other granulomatous diseases. Moreover, because tongue involvement may precede other signs of sarcoidosis, our findings indicate that when unexplained tongue involvement is noted, an evaluation for sarcoidosis should be made.
Department of Internal Medicine, Rouen University
Hospital, 76031 Rouen Cedex, France
Department of Stomatology, Le Havre Hospital,
Le Havre, France
Department of Histopathology, Le Havre Hospital,
Le Havre, France
email: isabelle.marie{at}chu-rouen.fr
References
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