British Dental Journal V205 N8

Orofacial presentations of IN BRIEF • Demonstrates the wide spectrum of orofacial manifestations of sarcoidosis PRACTICE above and beyond the ‘classical’ features sarcoidosis – a case series of the disease. • Serves as an aide memoire for junior hospital clinicians, general dental and review of the literature practitioners and dental students. • Highlights the dental aspects of this 1 2 3 4 systemic condition and is benefi cial when T. W. J. Poate, R. Sharma, K. A. Moutasim, M. P. Escudier formulating treatment plans for patients and S. Warnakulasuriya5 affected by the disorder. VERIFIABLE CPD PAPER Sarcoidosis is a multi-system disease of unknown aetiology characterised by the presence of non-caseating granulomas, the lungs and lymph nodes being the most affected sites. Orofacial manifestations of the condition are increasingly rec ognised, with several recent case reports where the initial presentation of the disease is in the region. Here, we report six cases of orofacial sarcoidosis which help to illustrate the wide spectrum of the condition. INTRODUCTION The lungs are often affected and in such prick in the distribution of all three Sarcoidosis is a systemic granulomatous cases the patient may present with a dry branches of the left trigeminal nerve. condition of unknown cause affecting cough, dyspnoea or chest pain. Other The left corneal reflex was also reduced. multiple organs.1 The disease is char common symptoms include a fl u-like The motor component of the nerve was acterised by the presence of non-case illness with fever, night sweats, fatigue spared and neurological examination ating granulomas and whilst any organ and arthralgia. Ocular discomfort and was otherwise normal. There was a 6 x 7 may be affected the most common sites reduced visual acuity due to uveitis mm firm swelling on the left lateral bor are the lungs and lymph nodes.2 More may occur as may lymphadenopathy der of the tongue. An excisional biopsy than 3,000 new cases of sarcoidosis are and enlargement of the liver and spleen. of the lingual swelling demonstrated the diagnosed in the UK each year with a Cutaneous lesions may also be present presence of non-caseating granulomas higher incidence among young adults and include a macular papular rash and suggestive of sarcoidosis (Fig. 1). Her aged 20–40.2,3 It occurs in all races but erythema nodosum.2 serum angiotensin converting enzyme appears to be more common in people of Oral involvement has been noted as (SACE) was raised at 110 U/L (normal African-American, Afro-Caribbean or the initial manifestation in several case level 27-82 U/L). Scandinavian origin.2,4,5 reports of sarcoidosis.6-8 A diagnosis of sarcoidosis was made It has been suggested that sarcoido We report six cases of orofacial sar and she was referred for chest and neu sis is due to an immunological response coidosis that have attended the oral med rology opinions. Clinical examination to an infective trigger in a genetically icine department in this dental institute of the chest was unremarkable whilst a predisposed individual, with the most in London in the past 24 months which chest X-ray revealed slight hilar promi likely candidates being mycobacteria help to illustrate the spectr um of the oro nence. Tuberculosis was excluded by and propionibacteria.5 facial presentations of this condition. the mantoux test. An MRI scan dem Affected patients may be asymp onstrated a mass adjacent to the left tomatic or have a wide range of non CASE 1: TRIGEMINAL NEUROPATHY trigeminal ganglion. Treatment was specific symptoms depending on the A 33-year-old Caucasian lady was commenced with prednisolone at a dose organs affected and stage of the disease. referred to the oral medicine department of 40 mg with appropriate prophylaxis by her general dental practitioner with including a bisphosphonate. a one year history of numbness of the At her subsequent review by a car left side of the face and forehead. She diologist it was felt that her cardiac 1Consultant in Oral Medicine; 2Senior House had also noticed an asymptomatic lump disease was probably a further mani Officer in Oral Medicine; 3*Speciality Dentist in Oral Medicine, Barts & The London NHS Trust, on the left side of her tongue which had festation of the sarcoidosis. Over the London; 4Consultant in Oral Medicine; 5Professor been present for the last three months. next four months her facial sensation of Oral Medicine, Dental Institute at Guy’s, King’s and St. Thomas’ Hospitals, London She had also recently been diagnosed improved. A repeat MRI showed the *Correspondence to: Dr Karwan Moutasim with hypertrophic cardiomyopathy but mass in the trigeminal ganglion to have Email: [email protected] had no other relevant medical history. reduced in size and her prednisolone was Refereed Paper She had no chest symptoms. therefore gradually reduced and then Accepted 11 July 2008 DOI: 10.1038/sj.bdj.2008.892 On examination she was found to have stopped over the following six months ©British Dental Journal 2008; 205: 437-442 reduced sensation to light touch and pin without relapse. BRITISH DENTAL JOURNAL VOLUME 205 NO. 8 OCT 25 2008 437 © 2008 Macmillan Publishers Limited. All rights reserved. PRACTICE CASE 2: SALIVARY AND LACRIMAL GLAND SWELLING A 43-year-old Indian female developed bilateral parotid and submandibular swelling. This was initially diagnosed by her general medical practitioner (GMP) as mumps and was managed conservatively. She subsequently developed a right sided lower motor neurone lesion of the sev enth cranial nerve for which she was pre scribed a two week course of prednisolone with good effect. However, three weeks after the cessation of the prednisolone the bilateral parotid and submandibular swelling recurred. On this occasion she was also noted to have bilateral lacrimal Fig. 1 High-power photomicrograph of lingual mucosa demonstrating non-caseating granulomas of sarcoidosis (case 1). An individual granuloma is shown in the left corner swelling. In view of this the GMP recom (haematoxylin & eosin) menced prednisolone and referred her to a local physician. Further investigations including liver function tests and SACE were within normal limits as were a chest X-ray and CT scan. In view of the per sisting salivary gland swelling she was referred to the oral medicine department for further investigation. On further questioning she reported dryness of the mouth and eyes. She also had arthralgia, weight loss and blurred vision which had worsened recently. She had no chest symptoms. On examina tion she had no swelling of the parotid, submandibular or lacrimal glands. She had slightly reduced visual acuity in the right eye and was noted to have ‘shotty’ cervical lymphadenopathy. Intra-orally she had a well lubricated and generally healthy oral mucosa. There was a good flow of clear saliva from all major sali vary gland duct orifices. A provisional diagnosis of sarcoidosis (Heerfordt’s syndrome) was made based on the his tory of salivary gland swelling, lac rimal involvement and seventh nerve palsy whilst the differential diagnosis Fig. 2 Anteroposterior chest radiograph showing bilateral hilar lymphadenopathy with borderline nodularity in case 2 included Sjögren’s syndrome. The unstimulated whole salivary flow and stimulated parotid salivary and submandibular glands which were leakage indicating activity in the optic flow rates were within normal limits. highly vascular. Parotid sialography nerve head. The patient declined any Schirmer’s test was negative for both was essentially normal with no delay of further investigations and a diagnosis eyes. Her autoimmune profi le includ emptying or sialectasis. A chest x-ray of salivary and ocular sarcoidosis was ing Ro and La antoantibodies was nega showed bilateral hilar lymphadenopathy made. In view of her persisting symp tive. The SACE was repeated and on this with borderline nodularity (Fig. 2) and toms oral prednisolone was continued occasion found to be raised at 94 U/L she was subsequently referred to a chest for two months before being reduced and (normal level 27-82 U/L). Ultrasound physician and ophthalmologist. Formal then stopped as her symptoms resolved. examination demonstrated an enlarged pulmonary function tests were normal She has since remained symptom free for jugulo-digastric lumph node and multi whilst a fluorescein angiogram showed two years with no further active treat ple hypoechoic areas within the parotid optic disc hyperfl uorescence and some ment required. 438 BRITISH DENTAL JOURNAL VOLUME 205 NO. 8 OCT 25 2008 © 2008 Macmillan Publishers Limited. All rights reserved. PRACTICE CASE 3: PAROTID GLAND at 69 IU/L (10-50 IU/L) and alkaline SWELLING phosphatase at 497 IU/L (30-130 IU/L). A 27-year-old gentleman of mixed eth The C-reactive protein (CRP) was raised nicity with a Caucasian mother and Afri at 98 mg/L (<5 mg/L). Blood cultures can father was admitted under the care were negative and HIV, hepatitis B of the neurologists with a three month and C and tuberculosis were excluded. history of generalised muscle aching Serology for toxoplasma, cytomegalo and weakness in his legs causing diffi virus, Epstein Barr virus and brucella culty in walking up stairs. He also had was unremarkable and an autoantibody bilateral swelling of the parotid glands screen was negative. SACE was raised at for which an oral medicine opinion 224 U/L (8-52 U/L). was requested. He had previously been Ultrasound examination demonstrated prescribed non-steroidal anti-infl am both parotid glands to be enlarged and matory drugs by his GMP which had inflamed with non-specific features of improved his symptoms over a period parotitis. Reactive lymph nodes were of six weeks. He then developed a fl u noted at the level of the carotid bifur like illness, fever, productive cough, cation bilaterally.

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