Facial sarcoidosis presenting as atypical facial pain.

In August 2003, a 45-year-old woman presented with a history of chronic facial pain. Limited computed tomography (CT) of the sinuses detected a 2.5-cm retention cyst in the right maxillary sinus (figure 1), and the patient was referred for endoscopic sinus surgery. Other symptoms included associated intermittent cough, postnasal drip, and sinus pressure. Her medical history was significant for perennial allergic rhinitis and gastroesophageal reflux disease, for which she took fexofenadine and rabeprazole, respectively. Her family history was significant for systemic lupus (mother) and borderline diabetes (father).

[FIGURE 1 OMITTED]

The physical examination was remarkable for a firm, slightly tender, nonmobile, subcutaneous, 1 x 1-cm mass that was fixed to the right midlateral nasal wall (figure 2). The condition of the overlying skin was unremarkable. Findings on flexible endoscopy were unremarkable, as were the results of the remainder of the head and neck examination. A fine-cut CT of the face detected a 9-mm soft-tissue nodule adjacent to the nose; the fat plane surrounding the nodule was normal, and there was no evidence of bony destruction, calcification, or surrounding infiltration (figure 3). Fine-needle aspiration biopsy was nondiagnostic, but it detected no malignant cells. The chest x-ray was unremarkable.

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In October 2003, the patient underwent excision of the paranasal mass, a right maxillary sinus antrostomy, and removal of her retention cyst. Pathology of the paranasal mass was significant for the presence of multiple, well-formed, noncaseating granulomas scattered in fibroadipose tissue and skeletal muscle, a finding consistent with sarcoidosis (figure 4). No fungal elements or mycobacteria were identified. The postoperative workup confirmed the diagnosis of disseminated sarcoidosis. The patient was treated with systemic steroids and a local triamcinolone injection, and her facial pain diminished.

[FIGURE 4 OMITTED]

In the absence of significant nasal complaints or objective endoscopic findings, chronic facial pain is rarely caused by sinus disease. The differential diagnosis includes sphenopalatine neuralgia, trigeminal neuralgia, midfacial segment pain, cluster headache, and atypical migraine. (1)

Sarcoidosis is a multisystem disease of unknown etiology that tends to affect young and middle-aged adults, primarily women. Blacks tend to be affected more than whites, and Japanese are affected more than other Asians. (2) Patients with sarcoidosis typically present with bilateral perihilar lymphadenopathy with pulmonary involvement and associated ocular or dermatologic lesions. Head and neck manifestations usually include cervical lymphadenopathy and parotid swelling with or without facial nerve palsy. A pattern of fever, uveitis, parotitis, and facial nerve palsy is known as Heerfordt's syndrome. (2) Lupus pernio (violaceous macules on the face) and flesh-colored papules near the eyes and/or nose may also be seen. Other skin conditions include onycholysis (separation of the distal nail from the nailbed), erythema nodosum (a tender nodule, usually on an extremity), and granuloma annulare-like lesions. (1,3)

Sinonasal symptoms of sarcoidosis are vague and extremely uncommon; they include nasal obstruction, postnasal drip, headache, and recurrent sinus infections. The nasal mucosa may be dry and friable; granulomas are less common. In rare cases of untreated advanced disease, a saddle-nose deformity may occur.

Identification of noncaseating granulomas on pathology in the appropriate clinical and radiographic setting usually confirms the diagnosis of sarcoidosis. Systemic steroids are the mainstay of therapy; methotrexate, cyclophosphamide, and antimalarial drugs have also been used.

References

(1.) Jones NS. Sinogenic facial pain: Diagnosis and management. Otolaryngol Clin North Am 2005;38:1311-25, x-xi.

(2.) Schwartzbauer HR, Tami TA. Ear, nose, and throat manifestations of sarcoidosis. Otolaryngol Clin North Am 2003;36:673-84.

(3.) Fawcett RS, Stulberg DL. The adult working years: Common skin findings in systemic diseases. Clinical Family Practice 2003;5: 667-89.

Lorraine Smith, MD, MPH, FACS; Ryan F. Osborne, MD, FACS

From the Cedars-Sinai Medical Group (Dr. Smith), and the Osborne Head and Neck Institute and the Head and Neck Cancer Center, Cedars-Sinai Medical Center (Dr. Osborne), Los Angeles.