Sarcoidosis rarely involves the head and neck region and can be a diagnostic challenge for the otorhinolaryngologist. Involvement of nasal mucosa was first reported by Boeck in 1905 [3
]. In patients with sarcoidosis, sinonasal involvement may develop, but the disease is rarely isolated to this area [4
]. McCaffrey and McDonald [5
] reviewed the records of 2319 patients diagnosed with sarcoidosis and found nasal mucosa involvement in 17 (less than 1%) of these cases. Wilson et al. [4
] noted nasal involvement confirmed by biopsy in 21 (2.8%) of 750 patients with sarcoidosis. A recent report by Zeitlin et al. [6
] stated a 4% incidence of nasal involvement in 159 patients with sarcoidosis. Those authors also noted that the actual incidence might be much higher. The most frequent sites of nasal involvement are the nasal septum and inferior turbinate, followed by the paranasal sinuses, nasal bone and cartilage, and subcutaneous tissues is the region [7
]. To the best of our knowledge, no cases of isolated sarcoidosis of the middle turbinates have been reported to date [2
]. Although there was no evidence of systemic involvement, this case might be an early manifestation of sarcoidosis, and should be followed-up for a long period.
The symptoms of nasal sarcoidosis are nonspecific. Nasal obstruction is the most frequent symptom, and others include postnasal drip, epistaxis, headache and recurrent sinus infections. Physical examination may reveal dry, friable mucosal lesions involving the septum and inferior turbinates, thick discharge and crusted polypoid tissues. Our patient's left middle turbinate was hypertrophic but no mucosal changes or crusting was observed during nasal endoscopy.
Histopathologic examination of a nasal biopsy is required for definitive diagnosis of nasal involvement of sarcoidosis. In some cases, this is the first evidence of systemic disease. Histologically, affected tissues exhibit multiple epithelioid cell granulomas that are organized collections of mature mononuclear cells. Hyaline fibrosis, leukocyte infiltration, necrosis, and refractile structures within epithelioid cells may also be present [8
]. The epithelioid cells secrete a lot of cytokines and mediators, including ACE [8
]. Thus, serum ACE level is elevated in 80% to 90% of patients with sarcoidosis [2
]. Our patient also exhibited elevated serum ACE. This sign is suggestive of sarcoidosis but is not a diagnostic biochemical marker. Further diagnostic tests are usually necessary to exclude other granulomatous disorders, such as tuberculosis, aspergillosis, actinomycosis, Wegener's granulomatosis, Churg-Strauss syndrome, lymphoma [3
]. In our case, we performed all indicated tests and examinations, and the results ruled out other granulomatous diseases.
In patients with sarcoidosis CT of the paranasal sinuses can show various types of lesions. These include multiple mucosal nodules (typically associated with various sinus opacities and inflammatory rhinitis), septal perforation, and destruction of nasal bone and/or cartilage [9
]. However, sites of opacification on CT are nonspecific radiologic findings, and accurate recognition of granulomas or nodules requires considerable expertise [3
]. Our patient's paranasal CT scan revealed bilateral concha bullosa but no evidence of sinusitis or any lesions in the nasal mucosa.
The clinical course of sarcoidosis is highly variable. There is a high incidence of recurrence, and a high rate (60%–70%) of spontaneous remission as well [1
]. Krepsi et al. [10
] proposed a staging system for sinonasal sarcoidosis. Patients in stage 1 have mild reversible disease without paranasal sinus involvement. Those in stage 2 have moderate potentially reversible disease with sinus involvement, and stage 3 is characterized as severe, irreversible disease. According to these descriptions, our patient had stage 1 sinonasal sarcoidosis.
Oral corticosteroids are the main treatment for systemic sarcoidosis [1
]. Cytotoxic agents (methotrexate and azathioprine), cyclophosphamide, chlorambucil, and anti-malarial drugs (chloroquine and hydroxychloroquine) are the alternative drug therapies for this patient group. The optimum treatment for nasal sarcoidosis depends greatly on the location and severity of the lesions. In patients with involvement of nasal structures or airways, topical or inhaled steroids can be used to avoid the complications that can occur with systemic corticosteroids [3
]. Use of additional intralesional steroid injections in cases of sinonasal sarcoidosis has also been reported [2
]. Systemic steroids are indicated in cases where symptoms are severe and the clinical course is highly destructive [3
Marks and Goodman [12
] suggested that surgery is indicated when medical treatment fails. They reported excellent short-term results and symptomatic improvement in all patients after surgery, but noted that the long-term results were less encouraging. Endoscopic sinus surgery (ESS) is effective for those few patients who develop nasal obstruction or chronic sinusitis due to anatomic blockage from sinonasal sarcoidosis lesions [13
]. Kay and Har-El [13
] concluded that although ESS does not eradicate the disease or prevent recurrence, it markedly improves quality of life by relieving symptoms and reducing the need for systemic steroids. Our patient underwent septoplasty and bilateral endoscopic concha bullosa resection via lateral laminectomies. She showed no disease progression and had no recurrence of complaints during 12 months of follow-up.
Nasal involvement of sarcoidosis is rare, but otorhinolaryngologists should consider this condition in differential diagnosis of sinonasal complaints. Nasal topical steroid application can control the progression of isolated nasal involvement in certain cases, and the surgery should be a last resort. Patients should be followed carefully over the long term since there is a tendency for recurrence and delayed systemic involvement.