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Home , Gingival enlargement, Herpangina, Orofacial granulomatosis

IN BRIEF • Lumps have a variety of causes, benign and malignant. • , imaging or other investigations are often indicated. 8

Oral Medicine — Update for the dental practitioner Lumps and swellings

C. Scully1 and D. H. Felix2

This series provides an overview of current thinking in the more relevant areas of for primary care practitioners, written by the authors while they were holding the Presidencies of the European Association for Oral Medicine and the British Society for Oral Medicine, respectively. A book containing additional material will be published. The series gives the detail necessary to assist the primary dental clinical team caring for patients with oral complaints that may be seen in general dental practice. Space precludes inclusion of illustrations of uncommon or rare disorders, or discussion of disorders affecting the hard tissues. Approaching the subject mainly by the symptomatic approach — as it largely relates to the presenting complaint — was considered to be a more helpful approach for GDPs rather than taking a diagnostic category approach. The clinical aspects of the relevant disorders are discussed, including a brief overview of the aetiology, detail on the clinical features and how the diagnosis is made. Guidance on management and when to refer is also provided, along with relevant websites which offer further detail.

ORAL MEDICINE This article discusses neck lumps, salivary gland . Children and young adults are 1. Aphthous and other swellings, and lumps and swellings in the . predominantly affected (Table 2). Enlarged cervi- common ulcers cal lymph nodes, especially in older people, may 2. Mouth ulcers of more NECK LUMPS also be related to malignant in the serious connotation The lymphoid system is the essential basis of drainage area (eg ) or may be a mani- 3. Dry mouth and disorders immune defences and comprises predominantly festation of systemic disease (eg HIV/AIDS). of salivation bone marrow, spleen, thymus and lymph nodes 4. Oral malodour too. Tissue fluid drains into lymph nodes which Examination of cervical lymph nodes act as ‘filters’ of and, after processing in Inspection of the neck, looking particularly 5. Oral white patches the nodes, lymph containing various immuno- for swellings or sinuses, should be followed by 6. Oral red and cytes drains from the nodes, to lymph ducts and careful palpation of the thyroid gland and all hyperpigmented patches then to the circulation. A consists of the lymph nodes, searching for swelling or 7. Orofacial sensation and a cortex, paracortex and medulla and is enclosed tenderness. movement by a capsule. Lymphocytes and antigens (if pres- The examination of lymph nodes in the neck is 8. Orofacial swellings and ent) pass into the node through the afferent lym- an important part of every orofacial examination. lumps phatics, are ‘filtered’ and pass out from the About one third of all the lymph nodes in the 9. medulla through the efferent lymphatics. The body are in the neck and dental surgeons can 10. Orofacial pain cortex contains B cells aggregated into primary often detect serious disease through examining it. follicles; following stimulation by these It is prudent to adopt a systematic and develop a focus of active proliferation (germinal methodical approach, examining different 1*Professor, Consultant, Dean, Eastman centre) and are termed secondary follicles. These lymph node groups in turn: Dental Institute for Oral Health Care follicles are in intimate contact with antigenpre- • Submental Sciences, 256 Gray’s Inn Road, UCL, University of London, London WC1X 8LD; senting dendritic cells. The paracortex contains T • Submandibular 2Consultant, Senior Lecturer, Glasgow cells, and the medulla contains T and B cells. • Pre-auricular/parotid Dental Hospital and School, 378 • Occipital Sauchiehall Street, Glasgow G2 3JZ / Associate Dean for Postgraduate Dental Causes of lymph node enlargement • Deep cervical chain. Education, NHS Education for Scotland, Many can present with in the 2nd Floor, Hanover Buildings, 66 Rose neck but the most common are lesions involving Both anterior and posterior cervical nodes Street, Edinburgh EH2 2NN *Correspondence to: Professor Crispian the lymph nodes (Table 1). should be examined as well as other nodes, liver Scully CBE Nodes enlarge in oral or local infec- and spleen if systemic disease is a possibility. Email: [email protected] tions in the drainage area (virtually anywhere in Most disease in lymph nodes is detected in the the head and neck). Most common is an enlarged anterior triangle of neck, which is bounded Refereed Paper © British Dental Journal 2005; 199: jugulo-digastric (tonsillar) lymph node, inflamed superiorly by the mandibular lower border, pos- 763–770 secondary to a viral upper respiratory tract teriorly and inferiorly by the sternomastoid

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drainage and nodes are then often firm, discrete Table 1 Causes of cervical lymph node enlargement and tender, but are mobile (lymphadenitis). The Inflammatory Infective Local Bacterial Local infections in the focus of can usually be found in head and neck the drainage area which is anywhere on the face, Viral Viral respiratory infections scalp and nasal cavity, sinuses, ears, and oral cavity. The local cause may not always Herpes zoster be found despite a careful search. For example, children occasionally develop a Staphylococcus Systemic Bacterial Syphilis aureus lymphadenitis (usually in a submandibu- Atypical mycobacterioses lar node) in the absence of any obvious portal of Cat scratch fever infection. Lymph nodes that are tender may be Brucellosis inflammatory, leukaemia or ; those Viral Glandular fever syndromes that are increasing in size and are hard may be (EBV, CMV, HIV, HHV-6) malignant. Lymph nodes may show reactive Protozoal Toxoplasmosis to a malignant tumour in the drainage area, or Others Mucocutaneous lymph node swelling because of metastatic infiltration. The syndrome (Kawasaki disease) latter may cause the node to feel distinctly hard, Non-infective and it may become bound down to adjacent Crohn’s disease tissues (‘fixed’), may not be discrete, and may even, in advanced cases, ulcerate through the Connective tissue diseases skin. The that frequently metastasise to cervical lymph nodes are oral squamous car- Primary Leukaemias cinoma (Article 9), nasopharyngeal carcinoma, tonsillar cancer and thyroid tumours. Secondary Metastases Usually one or more anterior cervical nodes Other Drugs, eg are involved, often unilaterally in oral neo- plasms anteriorly in the mouth, but otherwise not infrequently bilaterally. Table 2 Lymph node swellings at different ages More serious is the finding of an enlarged Decade Most common causes of swelling node suspected to be malignant but where the primary cannot be found. Nasopha- First Lymphadenitis due to viral respiratory tract infection ryngeal or tonsillar are classic causes of this and an ENT opinion should therefore be Second Lymphadenitis due to viral respiratory tract infection sought. Clinically unsuspected tonsillar cancer is Bacterial infection a common cause of metastasis in a cervical node Glandular fever syndromes of unidentified origin. Biopsy of the tonsil may HIV infection reveal a hitherto unsuspected malignancy. Toxoplasmosis Rare causes of cervical metastases include Third and fourth Lymphadenitis metastases from the stomach or even testicular Glandular fever syndromes tumours to lower cervical nodes. However, in HIV infection Malignancy some patients with a malignant cervical lymph node, the primary tumour is never located. After fourth Lymphadenitis Malignancy Generalised lymphadenopathy with or with- out enlargement of other lymphoid tissue such as liver and spleen (hepatosplenomegaly), sug- Table 3 Glandular fever syndromes gests a systemic cause. Lymph nodes may also swell when there are disorders involving the Features Adolescents and , fever, immune system more generally, such as the young adults lymphadenopathy mainly glandular fever syndromes, HIV/AIDS and relat- ed syndromes, various other viral infections; Causal agents Epstein-Barr Toxoplasma gondii Human immune (EBV) (CMV) deficiency (HIV) bacterial infections such as syphilis and tubercu- losis; and parasites such as toxoplasmosis. In the Investigations Paul-Bunnell Test CMV antibodies Sabin-Feldman HIV antibody titres systemic infective disorders the nodes are usual- EBV antibody titres dye test Lymphopenia Specific IgM T4 (CD4) cell numbers ly firm, discrete, tender and mobile. Lymph antibodies nodes may also swell in non infective lesions such as sarcoidosis; mucocutaneous lymph node muscle, and anteriorly by the midline of the syndrome; and neoplasms such as lymphomas neck. Nodes in this site drain most of the head and leukaemias (Table 1). In the latter instances, and neck except the occiput and back of neck. and in the glandular fever syndromes (where Lymphadenopathy in the anterior triangle of the there is lymphadenopathy often together with neck alone is often due to local disease, especially sore throat and fever; Table 3), there is usually if the nodes are enlarged on only one side. enlargement of many or all cervical lymph A limited number of lymph nodes swell usu- nodes and in some there is involvement of the ally because they are involved in an immune whole reticulendothelial system, with gener- response to an infectious agent in the area of alised lymph node enlargement (detectable clini-

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cally in neck, groin and axilla) and enlargement of the liver and spleen (hepatosplenomegaly). In the lymphomas particularly the nodes may be rubbery, matted together and fixed to deeper structures.

Management A medical opinion is often indicated.

Salivary gland swelling Salivary glands usually swell because of inflam- mation (), which is often viral but Fig. 1 Torus may have other causes (Table 4). Obstruction of mandibularis salivary flow is another common cause (obstruc- tive sialadenitis). Rare causes include salivary gland or other neoplasms. In children, most salivary gland swellings are caused by . In adults, most swellings of the salivary glands are caused by salivary duct obstruction (typically by a stone) but sialadeni- tis, Sjogren’s syndrome and neoplasms are important causes to be excluded.

Diagnosis of salivary gland swelling It can be difficult to establish whether a salivary gland is genuinely swollen, especially in obese Fig. 2 patients. A useful guide to whether the patient is simply obese or has parotid enlargement is to observe the outward deflection of the ear lobe which is seen in true parotid swelling. Diagnosis of the cause is mainly clinical but investigations such as imaging, liver function tests, serology for viral antibodies autoantibodies or biopsy, may be indicated.

Management A specialist opinion is usually needed and treat- ment is of the underlying cause. Fig. 3 Denture- induced hyperplasia Immediate treatment is needed for acute bac- and ulceration terial sialadenitis; under ideal conditions antimi- crobial therapy should be determined by results of culture and sensitivity of a sample of pus from the duct. However, as first line therapy, a penicil-

Table 4 Causes of salivary gland swelling Inflammatory Mumps Ascending sialadenitis Recurrent HIV parotitis Other infections (eg tuberculosis) Sjogren’s syndrome Fig. 4 Sarcoidosis Cystic Neoplasms (mainly pleomorphic salivary adenoma, but also monomorphic adenomas) Duct obstruction (eg ) Sialosis (usually caused by autonomic dysfunction in starvation, bulimia, diabetes, or alcoholic cirrhosis) Deposits rarely (eg amyloidosis and haemochromatosis) Drugs rarely (eg , methyl dopa, phenylbutazone, iodine compounds, thiouracil, Fig. 5 Calcium channel catecholamines, sulfonamides, phenothiazines and protease blocker-induced inhibitors) gingival swelling

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linase-resistant penicillin such as flucloxacillin is appropriate. In patients with penicillin , erythromycin is a suitable alternative. In addi- tion general supportive measures such as analge- sia and increased fluid intake are important. Thereafter, specialist referral is generally indicat- ed to identify any predisposing factors.

LUMPS AND SWELLINGS IN THE MOUTH Lumps and swellings in the mouth are common, Fig. 6 Human but of diverse aetiologies (Table 5) and some papillomavirus develop into ulcers, as in various bullous lesions infection (see article 2 ) and in malignant neoplasms (see article 9). Many different conditions, from benign to malignant, may present as oral lumps or swellings (see tables) including:

• Developmental; unerupted teeth, and tori - congenital bony lumps lingual to the mandibular premolars (; Fig. 1), or in the centre of the (torus palatinus; Fig. 2) are common causes of swellings related to the jaws. Fig. 7 Pyogenic • Inflammatory; is one of the most common causes of oral swelling. However, conditions characterised by chronic inflammation and , which can present with lumps or swellings — these include Crohn's disease, orofacial granulo- matosis (OFG), and sarcoidosis (discussed below). • Traumatic; haematoma may cause a swelling at the site of trauma. The flange of a denture impinging on the vestibular mucosa may stimulate a reactive irregular hyperplasia (denture-induced hyperplasia) (Fig. 3). • Neoplasms; benign epulides (Fig. 4) or malig- Fig. 8 Mucocoele in the nant tumours such as oral squamous floor of mouth () cell carcinoma (OSCC), Kaposi's sarcoma, lym- phoma and other neoplasms may present as swellings, as discussed in article 9. Occasion- ally, metastatic malignant disease may present as a lump. • Fibro-osseous lesions; fibrous dysplasia and Paget's disease can result in hard jaw swellings. • Hormonal and metabolic; may result in a gingival swelling (pregnancy epulis) • Drug-induced; a range of drugs can produce gingival swelling - most commonly implicated are phenytoin, calcium channel blockers and Fig. 9 Vascular hamartoma (Fig. 5). (haemangioma), dorsum of tongue • Allergic lesions; in particular can cause swellings. • Viral lesions; , common (ver- ruca vulgaris), and genital warts (condyloma acuminatum) are all among the lumps caused by human papillomaviruses (HPV; Fig. 6).

Causes of lumps and swellings according to site Carcinomas and other malignant neoplasms (See article 9) can present in any site. Fig. 10 Orofacial granulomatosis Gingiva showing cobblestoning Sometimes, hyperplasia is congenital. Rapidly buccal mucosa developing localised lumps, usually associated

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There are very few serious causes of gener- Table 5 Main conditions which may present as lumps or swellings in the mouth alised enlargements of the gingivae appearing spontaneously or rapidly, but leukaemia is one Normal anatomy Pterygoid hamulus Parotid papillae prime example. (foliate and circumvallate) Developmental Unerupted teeth Palate Odontogenic cysts Lumps of the hard palate may develop from Eruption cysts structures within the palate (intrinsic) or beyond Developmental cysts (eg thyroglossal, it (extrinsic). Thus, for example, torus palatinus dermoid) Haemangioma (Fig. 2) is an intrinsic bone , whereas a den- Lymphangioma tal abscess pointing on the palate (usually from Maxillary and mandibular tori the palatal roots of the first and second maxillary Hereditary gingival fibromatosis molars, or from upper lateral incisors) is extrinsic. Lingual thyroid Unerupted teeth, especially permanent canines, Inflammatory Abscess or second premolars are relatively common. Cellulitis Cysts Other causes of palatal swellings are uncommon Insect bites but it should be remembered that the palate is the Sialadenitis second most common site (after the parotid) for pleomorphic adenomas and other salivary neo- Chronic granulomatous disorders Orofacial granulomatosis plasms. Invasive carcinoma from the maxillary Crohn's disease sinus may produce a palatal swelling. Kaposi's Sarcoidosis sarcoma, typical of HIV/AIDS, may also present Traumatic Denture granulomata as a lump in the palate, or elsewhere. Developing Epulis unilateral hard palatal swellings, characteristi- Fibroepithelial polyp cally disturbing the fit of an upper denture in Haematoma Mucocele older patients, may denote Paget's disease. Surgical emphysema Neoplasms Carcinoma Floor of mouth Leukaemia Swellings in the floor of the mouth are more Lymphoma likely to arise from structures above the mylohy- Myeloma oid muscle than below it. The commonest Odontogenic tumours Minor salivary glands swellings in the floor of the mouth are denture- Others induced hyperplasia or a salivary calculus. Fibro-osseous Other lesions producing swellings in this Fibrous dysplasia area are a mucocele (known as ranula because Paget's disease of the resemblance to a frog's belly; Fig. 8) and Hormonal Pregnancy epulis/ neoplasms of the sublingual salivary gland Oral contraceptive pill gingivitis (usually malignant), but these are relatively Metabolic Amyloidosis uncommon. Patients occasionally describe a Other deposits lump which proves to be a swelling of the lin- Drugs Phenytoin gual aspect of the (more characteristic Calcium channel blockers of ameloblastoma than of dental abscesses or Ciclosporin cysts). Swellings of the submandibular salivary Allergic Angioedema gland and adjacent lymph nodes may occasion- Infective HPV ally be described by patients as being in the floor of the mouth. However, only very large Table 6 Main features of OFG swellings below the mylohyoid muscle are like- ly to produce a bulge in the mouth. Swellings in • Ulcers the floor of the mouth may inhibit swallowing • Facial or labial swelling and talking. • Angular Mandibular tori (Fig. 1) produce bony hard • fissures swellings lingual to the lower premolars. • Mucosal tags • 'Cobblestone' proliferation of the mucosa Tongue and buccal mucosa • Gingival swelling Discrete lumps may be of various causes – con- genital (Fig. 9; haemangioma), inflammatory, with discomfort, are most likely to be abscesses, traumatic or neoplastic. usually a dental abscess. Other localised The tongue may be congenitally enlarged swellings are usually inflammatory, such as the () in, for example, Down syndrome, pyogenic granuloma (Fig. 7) or neoplastic. or may enlarge in angioedema, gigantism, Most generalised gingival swellings are due to acromegaly or amyloidosis. hyperplasia with oedema related to plaque Causes of swellings include haematomas deposits, occasionally exacerbated by hormonal from trauma (such as occasional biting), infec- changes (, pregnancy) or drugs. Such tions, angioedema, fibro-epithelial polyps, changes often develop slowly – over weeks rather fibrous lumps, mucoceles (Fig. 9), vesiculobul- than days — and are usually without discomfort. lous lesions, and occasionally insect bites.

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syphilis and in malignant neoplasms. The flange of a denture impinging on the vestibular mucosa may stimulate a reactive irregular hyperplasia — the so-called denture granuloma or denture-induced hyperplasia (Fig. 3). Salivary neoplasms in the lip may sim- ulate, but are usually harder than, mucous cysts. Mucoceles are uncommon in the upper lip; discrete swellings there may well be sali- vary gland neoplasms.

Diagnosis of the cause of a lump or swelling Fig. 11 Orofacial granulomatosis with When patients refer to a lump in the mouth it is chronic lip swelling important to establish when it was first noticed. The tongue often detects even very small swellings and patients may also notice a lump because it is sore. Most patients have only a pass- ing interest in their but some examine their mouths out of idle curiosity, some through fear (perhaps after hearing of someone with ‘mouth cancer’). Indeed it is not unknown for some individuals (including dental staff!) to dis- cover and worry about the parotid papilla, foliate papillae on the tongue, or the pterygoid hamulus. The medical history should be fully reviewed, Fig. 12 and there should be a thorough examination, Neurofibromatosis, since some systemic disorders may be associated face with intra-oral or facial swellings (Fig. 12). Features of a lump which can be diagnosti- cally useful are: a) The number of lesions — particularly with regard to whether the lesion is bilaterally sym- metrical and thus possibly anatomical; b)Alteration in size; c) Any discharge from the lesion (clear fluid, pus, blood). d)When patients refer to a lump in the mouth, it is important to establish when it was first noticed.

Fig. 13 Important features to consider when making Neurofibromatosis, the provisional diagnosis of the cause of a lump upper lip or swelling include: • Position. The anatomical position should be defined and the proximity to other structures (eg teeth) noted. • Midline lesions tend to be developmental in origin (eg torus palatinus). • Bilateral lesions tend to be benign (eg sialosis — salivary swelling in alcoholism, diabetes or other conditions). • Most neoplastic lumps are unilateral.

Other similar or relevant changes elsewhere in the oral cavity should be noted. • Size. The size should always be measured and Fig.14 Minor salivary recorded. A diagram or photograph may be gland neoplasm helpful. • Shape. Some swellings have a characteristic Systemic conditions such as Crohn's disease, shape which may suggest the diagnosis: thus a orofacial granulomatosis and occasionally sar- parotid swelling often fills the space between coid may produce widespread irregular thicken- the posterior border of the mandible and the ing (cobblestoning) of the cheek mucosa (Fig. 10) mastoid process. or the (Fig. 11). • Colour. Brown or black pigmentation may be Some ‘lumps’ become ulcers, as in various due to a variety of causes such as a tattoo, bullous lesions, in primary and tertiary naevus or . Purple or red may be

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due to a haemangioma, Kaposi's sarcoma or (CT scans) or magnetic resonance imaging giant-cell lesion. (MRI), or ultrasound may, on occasions, be • Temperature. The skin overlying acute inflam- indicated. Photographs may be useful for matory lesions, such as an abscess, or a hae- future comparison. mangioma, is frequently warm. • Blood tests may be needed, particularly if • Tenderness. Inflammatory swellings such there is suspicion that a blood dyscrasia or as an abscess are characteristically tender, endocrinopathy may underlie the develop- although clearly palpation must be gentle ment of the lump. to avoid excessive discomfort to the • Biopsy is often required (Fig. 13) especially if patient. the lesion is single and chronic, since it may • Discharge. Note any discharge from the lesion be a neoplasm (Fig. 14) or other serious condi- (eg clear fluid, pus, or blood), orifice, or sinus. tion. • Movement. The swelling should be tested to determine if it is fixed to adjacent structures or CHRONIC GRANULOMATOUS CONDITIONS the overlying skin/mucosa — such as may be There are a number of patients who present with seen with a neoplasm. chronic swellings or lumps, which on biopsy • Consistency. Palpation showing a hard prove to have histological evidence of non- (indurated) consistency may suggest a carci- caseating epithelioid cell granulomas. These . Palpation may cause the release of fluid conditions include orofacial granulomatosis, (eg pus from an abscess) or cause the lesion to Crohn's disease, and sarcoidosis. blanch (vascular) or occasionally cause a blis- ter to appear (Nikolsky sign) or to expand. Orofacial granulomatosis Sometimes palpation causes the patient pain Orofacial granulomatosis (OFG) is an uncom- (suggesting an inflammatory lesion). The mon but increasingly recognised condition seen swelling overlying a bony cyst may crackle mainly in adolescents and young adults which (like an egg-shell) when palpated or fluctua- usually manifests with chronic facial and/or tion may be elicited by detecting movement of labial swelling, but which can also manifest with fluid when the swelling is compressed. Palpa- angular and/or cracked lips, ulcers, tion may disclose an underlying structure (eg mucosal tags, mucosal cobble-stoning, or gingi- the crown of a tooth under an eruption cyst) or val swelling (Figs 10 and 11; Table 6). show that the actual swelling is in deeper Some patients with similar features have, or structures (eg submandibular calculus). develop, gastrointestinal Crohn's disease or sar- • Surface texture. The surface characteristics coidosis. should be noted: papillomas have an obvious The aetiology of OFG is unknown but in some anemone-like appearance; carcinomas and there is a postulated reaction to food or other other malignant lesions tend to have a nodu- antigens (particularly to additives/preservatives lar surface and may ulcerate. Abnormal blood such as benzoates or cinnamaldehyde), or metals vessels suggest a neoplasm. such as cobalt. Most patients appear to develop • Ulceration. Some swellings may develop the problem in relation to dietary components superficial ulceration such as squamous cell such as chocolate, nuts, cheese or food additives. carcinoma. The character of the edge of the Conditions related to OFG include Miescher's ulcer and the appearance of the ulcer base cheilitis – where lip swelling is seen in isolation, should also be recorded. and Melkersson-Rosenthal syndrome – where • Margin. Ill-defined margins are frequently there is facial swelling with and associated with malignancy, whereas clearly recurrent facial palsy. defined margins are suggestive of a benign lesion. Diagnosis • Number of swellings. Multiple lesions suggest Diagnosis is clinical, supported by blood tests, an infective or occasionally developmental, endoscopy, imaging and biopsy to differentiate origin. Some conditions are associated with from Crohn's disease, sarcoidosis, tuberculosis multiple swellings of a similar nature, eg neu- and foreign body reactions. Specialist care is rofibromatosis (Fig. 12). usually indicated.

Investigations Management The nature of many lumps cannot be established Management is to eliminate allergens such as without further investigation. chocolate, nuts, cheese, cinnamaldehyde or food • Any teeth adjacent to a lump involving the additives and treat lesions with intralesional cor- jaw should be tested for vitality, and any ticosteroids or occasionally topical , caries or suspect restorations investigated. systemic clofazimine or sulfasalazine. • The periodontal status of any involved teeth should also be determined. Useful websites • Imaging of the full extent of the lesion and http://www.emedicine.com/derm/topic72.htm possibly other areas is required whenever lumps involve the jaws. OPT and special radi- Crohn’s disease ographs (eg of the skull, sinuses, salivary Crohn's disease is a chronic inflammatory idio- gland function), computerised tomography pathic granulomatous disorder. Many causal

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Patients to refer factors have been hypothesised but not proved. affects young adult females, especially Afro- Crohn's disease affects mainly the small intes- Caribbeans. Suspected malignancy including Lymphoma tine (ileum) but can affect any part of the gas- Sarcoidosis typically causes bilateral hilar trointestinal tract, including the mouth. lymphadenopathy, pulmonary infiltration and Suspected metastatic disease About 10% of patients with Crohn's disease impaired respiratory efficiency, skin and eye Unexplained lymphadenopathy of the bowel have oral lesions. Oral lesions lesions but can involve virtually any tissue. Orofacial granulomatosis may be seen in the absence of any identifiable Because of its vague and protean manifesta- gut involvement and are the same as those tions, sarcoidosis appears to be under-diag- seen in OFG — reddish raised lesions on the nosed. , or oral swellings gingiva, hyperplastic folds of the may be seen but sarcoidosis can involve any of (thickening and folding of the mucosa pro- the oral tissues and has a predilection for sali- ducing a ‘cobblestone type’ of appearance, vary glands, causing asymptomatic enlargement and mucosal tags), ulcers (classically linear of the major salivary glands and some have vestibular ulcers with flanking granulomatous xerostomia. The association of salivary and masses), facial swelling and . lacrimal gland enlargement with fever and There may also be features of gastrointestinal uveitis is known as uveoparotid fever (Heer- involvement such as abnormal bowel move- fordt's syndrome). ments, abdominal pain, rectal bleeding or weight loss. Diagnosis The most helpful investigations include: Diagnosis • Chest radiography (for enlarged hilar lymph Oral biopsy, haematological, gastrointestinal nodes) and other investigations may be required in sus- • Raised levels of serum angiotensin-converting pected Crohn's disease especially to exclude sar- enzyme (SACE) in acute disease coidosis. Specialist care is usually indicated. His- • A positive gallium or PET (positron emission tologically, the epithelium is intact but tomography) scan of lacrimal and salivary thickened, with epithelioid cells and giant cells glands surrounded by a lymphocytic infiltration. • Labial salivary gland biopsy (for histological evidence of non-caseating epithelioid cell Management granulomas). Topical or intralesional corticosteroids may effectively control the oral lesions but more fre- Management quently systemic corticosteroids, azathioprine or Patients with sarcoidosis but only minor symp- salazopyrine are required. toms often require no treatment. If there is active ocular disease, progressive lung disease, Sarcoidosis hypercalcaemia, or cerebral involvement or Sarcoidosis is a multi-system granulomatous dis- other serious complications, corticosteroids are order, of unclear aetiology, which most commonly given.

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