Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Otolaryngologic Manifestations of Sarcoidosis Sarcoidosis is a chronic, systemic granulomatous disease of unknown etiology. It generally affects people in their 20s to 40s and has a predilection for females, Blacks and Puerto Ricans. Worldwide, there is an increased prevalence of sarcoidosis in northern Europe, the southeastern United States, Australia, and Scandinavia. The etiology of sarcoidosis remains a mystery despite a great deal of research. An air-borne pathogen has been speculated, although a specific entity has never been identified. The immunologic response is probably initiated when an unknown antigen acts on a mononuclear phagocyte or T-helper cell, activating the T-helper cell to secrete Interleukin 2. This leads to further proliferation of the T-helper cells as well as secretion of a monocyte chemotactic factor responsible for recruiting the cellular building blocks needed for granuloma formation. Diagnosis of sarcoid is based on clinical findings, radiologic examination, serum chemistries and assays, skin testing, and ultimately on tissue diagnosis. Chest x-ray is abnormal in approximately 88 percent of patients with sarcoidosis, although this number drops to 50 percent when discussing patients presenting to otolaryngologists. The disease is staged based on chest radiographic findings as this has been found to have prognostic value. Patients with sarcoid exhibit a number of serologic abnormalities including elevated calcium, erythrocyte sedimentation rates, liver function studies and serum globulin. Angiotensin Converting Enzyme (ACE) is found to be elevated in between 60 and 80 percent of patients with sarcoid. Despite a number of false positives, elevated ACE levels are considered to be strong evidence for a diagnosis of sarcoidosis. Delayed hypersensitivity reactions reveal anergy in 86 percent of patients and are recommended in routine evaluation. The Kveim test, long considered the gold standard in diagnosis of sarcoidosis, is rarely performed today. Ultimate diagnosis depends on tissue pathology. This is most successfully accomplished by biopsy of obvious and easily accessible lesions. Other methods include mediastinoscopy and transbronchial biopsy. Blind biopsies of the tonsils, lip, conjunctiva and nasal mucosa have also been advocated by various researchers. Of these, biopsy of the minor salivary glands of the lip is the most promising and has been shown to have a yield of 50 percent in patients who exhibit abnormal chest radiographic findings. Excellent results have also been reported using FNA of palpable lymphadenopathy and salivary gland masses. Cervical lymphadenopathy is the most common head and neck presentation of sarcoidosis. Nodes are usually small, discreet and nontender and may wax and wane in size along the course of the disease. Lymph nodes are a frequent source of biopsy material for tissue diagnosis. In the absence of palpable lymphadenopathy, scalene fat pad lymph node biopsy was frequently performed in the past with a reported yield of 63 percent. Involvement of the nose may be either intranasal, extranasal or both. Intranasal disease prefers the septum and inferior turbinates. It is typified by punctate granulomatous lesions but is often represented only by crusting of the nasal mucosa, thickening of the septum and turbinates, rhinorrhea, and occasionally septal perforation. Anosmia is an occasional finding. Externally, the nose may become involved with dermatologic lesions including plaques, maculopapular eruptions, or lupus pernio, a disfiguring lesion characterized by a diffuse swelling of the nose and a purplish hue. Involvement of the major salivary glands occurs in about 6 percent of patients with sarcoidosis, This usually involves the parotid glands. Patients usually present with bilateral enlargement and dryness of the mouth. The glands are usually firm and non-tender and nodules may occasionally be felt. The associated ocular or facial nerve findings of uveoparotid fever are frequently encountered. Approximately 40 percent of patients will have spontaneous resolution of disease. Cranial nerve involvement occurs in approximately 5 percent of patients with sarcoidosis. The nerve most often affected is the facial nerve, followed by the 2nd, 10th and 8th nerve. Facial nerve paralysis occurs in 50 percent of patients with uveoparotid fever but may also occur in the absence of the other components of the syndrome. It is generally of a peripheral nature, although measurable changes in taste frequently place the lesion quite proximally. The histopathology is felt to consist of perivascular inflammation and granulomatous infiltration leading to focal ischemia and areas of neuronal infarct. Tenth nerve involvement may be of a central or peripheral etiology, arising from lesions at the base of the brain, or from pressure of enlarged paratracheal lymph nodes against the recurrent laryngeal nerve. Involvement is usually diagnosed from vocal cord paralysis. Otologic involvement is rarer still. This may present as hearing loss of a sudden, progressive or fluctuating nature, or as vestibular abnormalities. Various studies have shown both cochlear and retrocochlear lesions. The histopathology is also believed to consist of perivascular invasion with local ischemia. Although resolution occasionally occurs, the prognosis is not as good as with the facial nerve. Laryngeal involvement is found in approximately 1 percent of patients with sarcoidosis. The most common presenting symptoms are hoarseness and upper respiratory obstruction with stridor. Less common symptoms include dysphagia and cough. The epiglottis is the structure most often involved, although the disease may involve any part of the larynx. The appearance on laryngoscopy may consist of generalized erythema and edema, punctate nodules or mass lesions. The incidence of tonsillar involvement in sarcoidosis varies greatly in the literature, ranging from zero to nearly universal involvement in biopsy of patients with other manifestations of sarcoidosis. Involvement of the tonsils most frequently leads to tonsillar enlargement and may easily be mistaken for routine tonsillar hypertrophy. The mainstay of treatment for sarcoidosis is corticosteroids. Among the commonly recognized head and neck indications are airway obstructing lesions, neural involvement, and uveitis, although use of steroids has been recommended in various reports for virtually every manifestation of the disease. For most situations, treatment is with systemic oral corticosteroids, although intralesional injections of steroids have been successfully used for refractory laryngeal, dermatologic, intranasal or tonsillar lesions. Topical steroid sprays have also been recommended for intranasal sarcoid. Case Presentation A 60-year-old white female presented with a three year history of progressive hoarseness, which was more recently complicated by mild inspiratory stridor and dyspnea. She had been treated for several months by her allergist for presumed allergic symptoms before being referred to The Methodist Hospital for otolaryngologic evaluation. Flexible laryngoscopy revealed the presence of a large, smooth, mucosa-covered mass extending from the epiglottis down onto the left aryepiglottic fold which was nearly obstructing the larynx in a ball-valve fashion. There were also small mucosal irregularities extending from the mass down onto the false vocal fold. CT scan revealed a left aryepiglottic fold mass as well as left cervical lymphadenopathy. Chest x-ray was normal, and laboratory studies revealed an elevated globulin and normal calcium. Because of the patient's previous history of breast cancer a diagnosis of a metastatic nodule versus a laryngeal cyst was entertained. The patient was taken to the operating room where excisional biopsy of the mass was performed. Pathology revealed non-caseating granulomas. Stains and cultures for acid fast bacilli and fungi were negative. Skin testing revealed anergy. A presumptive diagnosis of sarcoidosis was made and she was started on corticosteroid therapy. Follow-up at eleven days and six weeks revealed steady regression of the remaining mucosal irregularities and the patient has had no other local or constitutional complaints. Bibliography Aggarwal AP, Jayaram G, Mandal AK. Sarcoidosis diagnosed on fine-needle aspiration cytology of salivary glands: a report of three cases. Diagn Cytopathol 1989;5:289-92. Babin RW, Liu C, Aschenbrener C. Histopathology of neurosensory deafness in sarcoidosis. Ann Otol Rhinol Laryngol 1984;93:389-93. Boeck C. Multiple benign sarkoid of the skin. J Cutan Genitourin Disease 1899:17:543-50. Bower JS, Belen JE, Weg JG et al. Manifestations and treatment of laryngeal sarcoidosis. 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