Introduction
Sarcoidosis is a disease of unknown aetiology first described in 1869. The disease most frequently affects young to middle-aged women and is characterised by non-caseating, epithelioid, granulomatous inflammation.Reference Newman, Rose and Maier1 Diagnosis is usually aided by detection of an elevated angiotensin-converting enzyme level and visualisation of characteristic bilateral hilar enlargement on a chest radiograph. However, there is currently no diagnostic test, and sarcoidosis is thus often a diagnosis of exclusion.Reference Newman, Rose and Maier1 The most common manifestation of sarcoidosis is pulmonary; however, cutaneous involvement occurs in up to 20 to 35 per cent of individuals with systemic sarcoid, and occasionally some patients present without any systemic disease.Reference Wilson and King2 Involvement and presentation varies with disease severity.
Otorhinolaryngological manifestations occur in approximately 10 per cent of patients diagnosed with sarcoid, but it is very rare for these to be the presenting feature.Reference Shah, White, Gooey and Hybels3 Nasal cutaneous presentations are uncommon, with only 0.3 to 4 per cent of sarcoid patients having this feature alone.Reference Zeitlin, Tami, Burgman and Winget4 Nasal sarcoid lesions usually comprise hardened, smooth, red (or sometimes violet) plaques.Reference English, Patel and Greer5 Patient symptoms range from polyps, through nasal congestion, rhinorrhoea and epistaxis, to severe nasal deformity.Reference Shah, White, Gooey and Hybels3 Nasal complications may arise, such as nasal obstruction leading to septal perforation. There is very little published experience with nasal reconstruction for this disease entity. In general, a cautious, conservative attitude prevails out of fear of complications or failure.
We present the use of subcutaneous triamcinolone injections to treat severely evolving nasal deformity secondary to sarcoidosis.
Case report
A 30-year-old woman with a long-standing history of sarcoidosis, in addition to asthma, presented to a tertiary referral rhinology clinic complaining of breathing difficulty and nasal deformity.
Flexible nasoendoscopy revealed red nasal plaques typical of nasal sarcoidosis, together with significant widening of the nasal bridge.
This patient was treated with local steroid injections in the form of triamcinolone (Kenalog 40 mg/ml) injected both intralesionally and subcutaneously over the nasal dorsum; this was repeated three and eight months later. The patient was followed up two, six and 12 weeks after each injection. This treatment significantly improved the patient's breathing, and there was long-lasting improvement in the nasal shape (Figures 1 to 4). It was acknowledged that further injections may be needed in the future.
Clinical photographs showing (a) pre-treatment and (b) post-treatment views, viewed from above. Published with patient's permission.
Clinical photographs showing (a) pre-treatment and (b) post-treatment lateral views. Published with patient's permission.
Clinical photographs showing (a) pre-treatment and (b) post-treatment oblique views. Published with patient's permission.
Clinical photographs showing (a) pre-treatment and (b) post-treatment views, viewed from below. Published with patient's permission.
Discussion
Sarcoidosis consists of epithelioid granulomas interspersed with lymphocytes. In patients with nasal manifestations, pre-intervention, unenhanced computed tomography reveals widespread nasal inflammation and an abnormal soft tissue mass. Nasal deformity induced by this condition is a result of the combined effect of cutaneous and mucosal disease.
• Sarcoidosis is characterised by non-caseating granulomas of unknown cause
• Manifestations are usually pulmonary, sometimes cutaneous and rarely nasal
• Nasal sarcoid can cause polyps, congestion, rhinorrhoea, epistaxis and nasal deformity
• Published treatments include systemic immunosuppressants and surgery (including laser)
• Triamcinolone injections successfully treated the presented case
Surgery has been shown to successfully treat individuals with nasal granulomatosis refractory to pharmacological treatment.Reference Preminger, Hiltzik, Segal and Morrison6 However, problems with surgical treatment include the lack of satisfactory post-operative cosmesis and the risk of skin graft loss.
Laser has also been used for sarcoidosis treatment, with beneficial, long-lasting effects.Reference Baughman and Lower7, Reference Carlesimo, Giustini, Rossi, Bonaccorsi and Calvieri8
Pharmacological agents which are known to have measurable success in treating persistent, widespread sarcoidosis include allopurinol, infliximab, hydroxychloroquine, methotrexate and retinoids.Reference Newman, Rose and Maier1, Reference Young, Chalmers, Griffiths and August9, Reference O'Donoghue and Barlow10
Triamcinolone is a long-acting, synthetic corticosteroid given orally, by injection (40 mg/ml, under local anaesthesia if needed), by inhalation, or as a topical ointment or cream.
Topical triamcinolone injections provide an alternative modality of management for chronic, evolving inflammatory conditions in patients in whom surgery is undesirable and systemic medical treatment has been unsuccessful. Patients should be followed up two, six and 12 weeks after injections. Further application of this treatment may encourage its use as a long-term, conservative therapeutic option.
Conclusion
In the presented case, nasal sarcoidosis was treated with local triamcinolone injection, with a successful aesthetic and functional outcome. Treatment was quick, simple and relatively cost-effective, with little or no co-morbidity. In this case, an improvement in quality of life comparable to that following formal surgical rhinoplasty could be expected.
Acknowledgement
Many thanks to our patient who kindly consented to have her case published.
