The tongue in clinical diagnosis

Yucel A, Akman A, Denli YG et al. A case of systemic amyloidosis associated with multiple myeloma presenting as macroglossia and purpura. JEADV 2004; 18: 378–379 The case report of Yucel et al. illustrates the importance of the tongue in clinical diagnosis.1 The 53-year-old man suffered from macroglossia and purpura of the tongue and lips as a sign of myeloma-associated systemic amyloidosis. The woody, hard, enlarged tongue with purpura (fig. 1) is a classical sign of amyloidosis and a stimulus to seeking the cause for the amyloid deposition. Amyloidosis: the tongue is thickened and enlarged (macroglossia); it is woody and hard to palpation and shows purpura. Examination of the tongue can provide clinical clues to several conditions with systemic import as well as local abnormalities that may be recognized by the patient in self-examination. Recognition of tongue abnormalities as benign or as a sign of systemic disease is a valuable clinical skill. It is important for the clinician to know and recognize the spectrum of disorders affecting the tongue. A thorough examination of the tongue is an integral part of the physical examination and may provide clues to systemic disorders. A systematic approach to the examination, beginning with inspection of all aspects of the tongue (dorsum, lateral margins and ventral surface) and concluding with palpation of the entire surface from the tip proximally, provides a thorough and complete examination. Note the shape and colour of the tongue as it rests in the mouth. The presence of notching on the lateral margins of the tongue is indicative of macroglossia or a tongue-thrusting habit as the pressure from the teeth on the tongue creates the notches. The normal tongue is pink in colour, and the papillae easily visualized. Many conditions present with alterations in colour or texture of the tongue, and clinicians should be familiar with the normal appearance to best appreciate changes to normal architecture.2,3 The furred tongue is a common benign entity caused by hyperplasia of the filiform papillae and retention of keratin debris. Clinically, a complete or partial white coating of the anterior tongue gives the appearance of thin white fur that gives the condition its name (fig. 2). The furring may vary in colour from white to brown. Particularly in smokers, the fur may take on a tan colour. The condition is seen commonly in otherwise healthy individuals who are smokers, mouth breathers, or have poor oral hygiene. Patients on a soft diet lacking roughage may also develop fur. In the ill patient, the furred tongue is usually due to dehydration or fever. The furred tongue is usually asymptomatic, although halitosis can result from degradation of food and other debris trapped within the plaque of fur. Furred tongue: the dorsum of the tongue is covered by a white coating or 'fur', consisting of keratin, debris, bacteria and food particles. This material contributes to halitosis. The diagnosis is based on clinical findings, and biopsy is therefore not necessary. Smoking cessation, rehydration, resolution of febrile illness, and correction of mouth-breathing, respectively, are important management steps in otherwise well patients. Treatment includes increasing roughage and fibre in the diet to promote desquamation. Brushing the tongue with 5–15 strokes daily using a soft-bristled toothbrush and dentifrice is helpful. The black hairy tongue is a dark brown to black tongue that may also appear 'hairy'. As with the furred tongue, the involved area is usually the anterior two-thirds of the dorsal tongue. The filiform papillae are elongated with pointed ends and have a brown to black coating thus appearing to be covered with thick brown–black 'hair'. The dark colour results from chromogenic bacteria that become trapped between the hyperkeratotic papillae. The prevalence has been reported as 3–4% of the population.3,5 Most patients are asymptomatic but some experience halitosis, abnormal taste or nausea, presumably due to the elongated papillae. Smoking and poor oral hygiene can be causative. Long-term use of antacids containing bismuth or oxidizing mouthwashes are other causes. The black, hairy tongue may occur after a course of broad-spectrum antibiotics that perturb the normal oral microfloral balance. Biopsy is not necessary for diagnosis. Treatment involves brushing the tongue with 1–2% hydrogen peroxide or dentifrice. As with the furred tongue, increasing roughage, correction of mouth-breathing, and smoking cessation are important therapeutic interventions. Avoiding further antibiotics allows the normal flora to reconstitute, diminishing the prevalence of the pigment-producing microflora. The fissured tongue presents as a short or long deep central groove on the dorsal tongue with multiple irregular side grooves. The fissures can occur on the lateral margins. The papillae are present in the superficial zone of the fissures, but the deeper portions may be depapillated, contributing to bacterial overgrowth and inflammation. The fissured tongue is normal with ageing. It is the most common developmental defect of the tongue.4 Prevalence has been reported in dental studies to be 5–11%. The fissures are typically asymptomatic unless inflamed due to trapped food debris, bacterial overgrowth and low-grade infection. The fissured tongue has been associated with Down's syndrome, acromegaly, Sjogren's syndrome, pustular psoriasis, psoriasis vulgaris, geographical tongue and Melkersson–Rosenthal syndrome (fig. 3). The fissured tongue is often an incidental finding. It is a common condition, particularly in older patients, and unless the history suggests otherwise, no additional evaluation is needed. Fissured tongue: the dorsum of the tongue is thrown into deep folds ('fissures'). The folds trap debris and food particles, contributing to halitosis. The tongue is also scalloped along the lateral borders indicating macroglossia in this patient with the Melkersson–Rosenthal syndrome. The condition is diagnosed clinically. Patients should be reassured of its benign nature. Treatment involves brushing the tongue (5–15 strokes) with dentifrice and a soft-bristled toothbrush after meals and before sleeping to prevent a build-up of food debris and bacteria in the fissures, which can cause halitosis. If pain is a problem, a topical anaesthetic such as viscous lidocaine can be applied prior to meals. Sublingual varices are benign vascular dilatations. Patients may note a discoloration of the ventral or lateral tongue. The lingual veins become dilated and tortuous (fig. 4). The varices are usually asymptomatic and are noted incidentally by the patient. Ten percent of the population over the age of 40 are affected. No clinical significance has been established. No treatment but reassurance is necessary. However, the clinician should be certain to exclude hereditary haemorrhagic telangiectasia (Osler–Weber–Rendu syndrome). The blue rubber bleb nevus syndrome and the superior vena cava syndrome should also be considered but are easily excluded with a thorough clinical evaluation. Sublingual varices: dilated or tortuous vessels are present on the ventral and lateral tongue surfaces. Ten percent of patients > 40 years of age have these varices. Geographic tongue has multiple synonyms including benign migratory glossitis, erythema migrans and glossitis areata migrans. Patients present with red and white patches on the dorsum of the tongue. The red patches lack papillae and are atrophic while the white areas have either normal or hyperplastic papillae. The patches are irregular and sharply demarcated resembling a map, hence the name (fig. 5). Patches may occur on the lateral margins or ventral tongue or rarely elsewhere in the mouth (geographical stomatitis). Characteristically, the plaques vary in location and shape hourly to daily. Prevalence ranges from 1 to 14%, affecting all age groups and females more often than males. A familial history of this disorder may be present. This benign inflammatory disorder is usually asymptomatic unless fissures are present. Forty percent of patients with geographical tongue have fissured tongue. Geographic tongue has been associated with atopy and psoriasis vulgaris although it usually occurs as an isolated abnormality.5,6 Geographic tongue. Note the atrophic red patches adjacent to the hypertrophic, white furred patches. The plaques change size and shape over hours to days. The atrophic patches are tender. Treatment is reassurance. If patients are experiencing discomfort with spicy, sour or salty foods, these should be avoided. Palliative therapy with anti-yeast treatment, topical corticosteroids or topical analgesics may be helpful. Median rhomboid glossitis has also been called chronic candidiasis and central papillary atrophy of the tongue. It is uncommon with a prevalence < 1%.4,5 Men are affected three times more often than women. The condition appears as a rhomboid-shaped plaque in the mid-third of the tongue with surface changes of hypertrophy or atrophy (fig. 6). Patients may complain of a burning sensation when eating spicy foods, although most are asymptomatic. The aetiology is uncertain. Until recently, it was thought to be due to congenital persistence of the tuberculum impar, although in general the changes are not present at birth. It has been associated with chronic Candida infection, with one author reporting 90% of patients with median rhomboid glossitis demonstrating candidal infection.7,8 Some clinicians or patients may be concerned about the possibility of oral cancer. If this is a significant concern, a biopsy to exclude squamous cell carcinoma may be indicated. Median rhomboid glossitis. A rhomboid plaque is present in the middle third of the dorsal tongue. The plaque may be atrophic, erosive, hypertrophic or ulcerative. When median rhomboid glossitis is found in association with palatal inflammation corresponding to contact with the involved area on the tongue, immunosuppression should be suspected. This condition is called candidal infection of the tongue and non-specific inflammation of the palate (CIT-NIP). It has been considered a thumbprint of AIDS so HIV status should be sought in these patients.9 The smooth tongue presents as a smooth, glossy, tender tongue. Patients usually complain of a burning sensation or a sore tongue. The tongue lacks the normal rough appearance created by the papillae. The background colour may be red, pink or magenta (fig. 7). This condition is the manifestation of a systemic disorder such as malabsorption or nutritional deficiencies of iron, folic acid, vitamin B12, riboflavin or niacin.2,3,10 Correction of the nutritional deficiency results in rapid regeneration of the papillae. The smooth tongue can also be a manifestation of syphilis infection, amyloidosis, gluten-sensitive enteropathy or cardiac failure. Sjogren's syndrome, Plummer–Vinson syndrome and Riley–Day dysautonia syndrome can present with smooth tongue. Smooth tongue: this is an atrophic tongue with loss of papillae. It may be red, pink or magenta in colour. The tongue is tender, sore or burns. This patient also has a fissured tongue. Treatment involves correction of an underlying systemic cause if possible. Symptomatic treatment with a soft, bland diet is necessary as the patients usually complain of a painful, sore tongue. Oral hairy leukoplakia (OHL) is the term used to describe a benign white lesion on the lateral margins of the tongue.11 It is traditionally seen in immunosuppressed patients infected with Epstein–Barr virus (EBV). OHL presents as white, linear, hairy plaques on the lateral margin of the tongue and/or buccal mucosa. Early lesions appear corrugated because there are white plaques on the ridges of the lateral tongue and erythematous mucosa in the troughs created by muscle attachments. With time, the lesions become completely white. OHL has been reported almost exclusively in immunodeficient patients. Originally it was believed to be limited to homosexual HIV-positive males, but OHL has been reported in other HIV patients and in patients with other causes of immunodeficiency such as organ transplant recipients. It has been proposed that identification of EBV DNA by in situ polymerase chain reaction be used for diagnosis as in one study a 17% false positive rate was found by using clinical criteria alone.12 If oral hairy leukoplakia is found in patients without known immunosuppression, HIV testing and an evaluation for other immunosuppression states should be pursued. Herpetic geometric glossitis is found in immunosuppressed patients with chronic low-grade herpes simplex virus infection of the tongue.13 Patients present with tender linear fissures on the dorsal tongue. Often the fissures have a striking geometric pattern with right-angle radiation. This condition has been reported in immunocompromised hosts and is attributed to chronic herpes simplex virus infection as the glossitis responds to treatment with oral antiviral therapy. Macroglossia is enlargement of the tongue out of proportion to the size of the jaws. The condition may be congenital or acquired. The tongue will appear large within the oral cavity. The lateral margins are scalloped from the constant pressure against the teeth (fig. 8). If a few teeth are missing, the tongue may expand into the available space to produce a pseudo-tumorous appearance. Haemorrhage may be evident if the enlargement is sufficient to interfere with talking or mastication with inadvertent tongue biting. The tongue may have a hard, woody, indurated feel to palpation (fig. 1). Many associations including Down's syndrome, amyloidosis and hypothyroidism should be considered, as well as infectious diseases such as blastomycosis, histoplasmosis, tuberculosis, actinomycosis and other infiltrative disorders (Table 1). Amyloidosis may present with macroglossia and pinch purpura (purpura following trauma).1 Macroglossia. The enlarged tongue may have scalloped borders from constant pressure against the teeth. If a few teeth are missing, a pseudotumour develops in the available space. The burning mouth syndrome (BMS) occurs when patients suffer constant oral pain. Clinically, a normal oral mucosal examination may be present. BMS is a diagnosis of exclusion. BMS has been characterized as a psychosomatic disorder occurring in postmenopausal women. However, recent studies have suggested a multifactorial aetiology with local, systemic and psychological/psychiatric factors at play contributing to the symptom complex.14,15 Clinically, patients will describe a burning, tingling, painful, sore or scalded sensation of the tongue, usually involving the anterior two-thirds and tip of the tongue. Other oral sites may be affected. Most commonly, multiple causes for BMS can be identified. Treatment must be addressed simultaneously for all causes to achieve the best control of symptoms. Four major aetiological categories are 1) systemic, 2) local, 3) psychiatric/psychological and 4) idiopathic factors.15,16 Systemic factors include nutritional deficiencies. Anaemia and deficiencies of iron, zinc, vitamins B12 and B6, and folate should all be excluded. Replacement therapy is recommended in patients with documented deficiency. Other systemic factors include disorders such as diabetes mellitus as well as the menopausal state.14 Reflex oesophagitis has also been implicated as a cause of BMS. Local factors include xerostomia, allergic contact stomatitis, denture-related problems and candidiasis. Xerostomia is a frequent complaint among BMS patients and may itself be caused by drugs (such as tricyclic antidepressants, antihypertensives and antihistamines), local irradiation, age or connective tissue diseases such as Sjogren's syndrome. Denture-related problems are usually due to faulty design. Irritation, candidiasis or parafunctional habits cause the denture-sore mouth rather than a true allergic response to denture material.14–16 Oral candidiasis may be a causative factor in a significant number of patients with BMS. Oral candidiasis may arise from xerostomia, corticosteroid treatment, dentures or antibiotic therapy. Although Candida is a normal constituent of the oral mucosa in many people, overgrowth may produce symptoms. Empirical treatment is often prescribed to patients with BMS with benefit. Allergic contact stomatitis is a controversial cause of BMS as the role of allergens is not clear. However, as flavourings and food additives have been implicated in BMS, patch testing with specific oral flavours and preservatives in patients with refractory BMS can be valuable. Allergies to cinnamon aldehyde, ascorbic acid, tartrazine, benzoic acid, propylene glycol, menthol and peppermint have all been identified as causes of mouth pain.15,16 Psychiatric disease is a common factor in patients with BMS, and many patients have an underlying psychiatric diagnosis, commonly depression or anxiety. Cancer phobia may also be present. Many patients are so frustrated with their persistent symptoms that they become anxious and/or depressed. Patients frequently present complaining of tongue abnormalities. Knowledge of normal tongue anatomy and architecture will enable the clinician to differentiate variations of normal from abnormal conditions. Many tongue conditions are benign and require reassurance and explanation, with little to no treatment. Others can signify systemic disorders. Therefore, examination of the tongue is an integral part of a complete physical examination. Recognizing the disorders of the tongue that are benign and do not require treatment or further evaluation will prevent unnecessary testing for the patient. On the other hand, careful evaluation of the tongue may provide valuable clues to a systemic disorder including myeloma-associated systemic amyloidosis.1