An oral lesion as the primary clinical manifestation of sarcoidosis

Abstract

An oral lesion as the first clinical presentation of sarcoidosis is extremely rare. We present the case of a 39-year-old woman who was referred to the oral and maxillofacial surgery department with a persistent asymptomatic nodular lesion in the hard palate. This was located adjacent to a grossly carious upper first molar and a provisional diagnosis of chronic periapical granuloma was made. An incisional biopsy of the lesion was carried out, which unexpectedly revealed the presence of a non-caseating granulomatous inflammatory reaction. A referral was sent to the respiratory medicine team and a diagnosis was later confirmed of stage II sarcoidosis. This case report highlights the need for clinicians to be aware of all possible causes of oral lesions, including rare manifestations of underlying systemic disease.

Sarcoidosis is a multisystemic disease of unknown aetiology that is characterised predominantly by non-caseating granulomas in the lungs and lymph nodes.¹ It is estimated to affect 1 in 10,000 people in the UK, most commonly in young adults aged 20–40 years.² Patients frequently present with symptoms of dry cough, shortness of breath and joint pain. Only 10–15% of patients present with head and neck manifestations, and so oral involvement of this disease (particularly as the first clinical presentation) is extremely rare.³

Case history

A 39-year-old woman was referred to the oral and maxillofacial surgery department by her general dental practitioner owing to the presence of a swelling in the hard palate resembling ‘multiple very small mucocoeles’. This lesion had been persistent for at least four weeks, with no history of painful symptoms. Medically, the patient was fit and well with no known drug allergies. She was an ex-smoker, having stopped ten years previously, with a four pack-year history. She consumed an average of four units of alcohol per week and was a housewife.

Extraoral examination was unremarkable. Intraorally, a 10mm × 4mm lesion was noted in the posterior upper left hard palate consisting of multiple well defined, soft, asymptomatic, violaceous nodules. This lesion was located adjacent to a grossly carious upper left first molar tooth. Panoramic radiography revealed a large periapical radiolucency around this tooth (Fig 1). A chronic periapical granuloma of the upper left first molar was the provisional diagnosis. However, an incisional biopsy under local anaesthesia was arranged owing to the atypical appearance of the lesion and the patient also returned to her general dental practitioner to have the upper left first molar extracted. The lesion persisted following the extraction.

Figure 1.

Panoramic radiography revealing a grossly carious upper left first molar with periapical radiolucency. The poor prognosis of both lower second molars was also noted.

Unexpectedly, the histopathology report described the presence of a non-caseating granulomatous inflammatory reaction. Ziehl–Neelsen and Grocott stains were negative. The histopathology report advised correlating the patient’s signs and symptoms with the clinical features of sarcoidosis. In light of these histological findings, a referral was made to the respiratory medicine team.

The patient was subsequently seen by a respiratory physician and on further questioning, she did reveal symptoms of cough for the last month and mild shortness of breath on exertion. She reported no family history of tuberculosis and had not recently travelled abroad. On examination, there was absence of clubbing, rash, lymphadenopathy and joint swelling. A chest x-ray showed a granulomatous lesion in the upper left lobe of the lung (Fig 2). Computed tomography of the chest revealed evidence of several mediastinal and hilar lymph nodes as well as multiple soft tissue lesions in the right and left lungs, mostly localised to the mid and upper lung fields. In order to confirm a diagnosis of sarcoidosis, endobronchial ultrasonography guided transbronchial needle aspiration was carried out, which correlated with the results from the incisional biopsy of the hard palate, revealing poorly formed granulomas and lymphocytes, and no evidence of malignant cells.

Figure 2.

Chest x-ray showing a granulomatous lesion in the upper left lobe of the lung

A diagnosis was made of stage II sarcoidosis with pulmonary and palatal involvement. In view of her cough symptoms and pulmonary lesions, the patient was placed on prednisolone (30mg daily for two months). At the subsequent review appointment, she stated that her symptoms of cough had greatly improved and the swelling in her palate had significantly reduced in size such that it was no longer touching her tongue. As her symptoms had significantly improved, she was tapered off the steroid completely. She is now being kept under annual review with the respiratory medicine team.

Discussion

Sarcoidosis is a multisystemic granulomatous disease presenting most commonly in the 20–40-year age group with symptoms of shortness of breath and persistent dry cough along with rash and joint pain.² The majority (90%) of patients present with pulmonary manifestations while 25% present with cutaneous, and 10–15% with head and neck manifestations.³ A recent literature review found only 68 well documented cases of oral sarcoidosis since Schroff reported the first case in 1942.³

Clinical features of sarcoidosis in the head and neck region are highly variable. Lesions in the soft tissues of the oral cavity commonly present as persistent, firm, asymptomatic nodules or ulcers affecting the lips, tongue, hard/soft palate, buccal mucosa or gingivae; this may also be accompanied by generalised gingival hyperplasia, gingivitis or gingival recession.¹ Sarcoidosis can manifest in the jaw bones as rapid loosening of teeth with severe alveolar bone loss, mandibular swelling or a non-healing socket. There are also reports of sarcoidosis involvement in the salivary glands with presentation of a persistent, non-tender, firm swelling (usually bilateral) and accompanying xerostomia.^1,4

Diagnosis of sarcoidosis is made by a combination of clinical presentation and histopathology although other granulomatous conditions such as tuberculosis and orofacial granulomatosis need to be excluded. Management of this condition can range from no treatment to systemic corticosteroid therapy with possible surgical excision and curettage of localised oral lesions.³

Conclusions

Although extremely rare, oral lesions may be the initial manifestation of sarcoidosis and can pose diagnostic difficulties, as experienced in our case. This report highlights the need for clinicians to take a holistic approach to the management of every patient and to investigate all possible causes of oral lesions including underlying systemic disease. If necessary, patients should be referred promptly to the appropriate specialty for management to prevent further systemic involvement.