An unusual case of external ear inflammation caused by sarcoidosis - Original Article

Ear, Nose & Throat Journal, Dec, 2003 by Emer E. Lang, Zaruk Jamilla El, Michael P. Colreavy, Susan Kennedy, Helena Rowley, Conrad Timon

Abstract

Inflammation of the pinna can occur in conjunction with polychondritis and otitis externa. We describe a case of pinneal inflammation that proved to be sarcoidosis, and we discuss the otolaryngologic manifestations of sarcoidosis.

Introduction

Sarcoidosis is a common multisystem disorder of unknown etiology that can mimic disease processes such as polychondritis.(1) It can be self-limiting or progressive and relentless. Lesions are made up of noncaseating epithelioid and giant-cell granulomas.

Sarcoidosis can affect several organs and tissues, most commonly the hilar lymph nodes, eyes, skin, and mucosal surfaces. Otolaryngologists might encounter lesions that affect the nose, tonsils, larynx, trachea, nasopharynx, facial bones, and paranasal sinuses. (2,3) The ear is an uncommon site, although involvement of the acoustic nerve manifesting as sensorineural deafness has been documented. (4) Moreover, sarcoidosis has been described at the site of earlobe piercings, usually in patients with systemic sarcoidosis. (5) Sarcoidosis with external ear manifestations is exceedingly rare, but it should be considered in the differential diagnosis of an acutely inflamed external ear. In this article, we describe a case of pinneal sarcoidosis.

Case report

A 38-year-old man came to the accident and emergency department of the Royal Victoria Eye and Ear Hospital in Dublin with an acute exacerbation of swelling of the right pinna, which had been present for 4 months. Initially the swelling was painless, but it had become acutely tender during the preceding week. No associated otologic symptoms were noted. The patient complained of feeling generally unwell, and he had noticed a significant weight loss over the preceding weeks.

Examination revealed that the helical cartilage and pinna on the fight side was inflamed and thickened and that the overlying skin was erythematous and excoriated (figure 1). The ENT examination was otherwise normal. A blood test revealed that the patient's serum angiotensin-converting enzyme (ACE) level was elevated, but other parameters were normal. Autoimmune antibody testing was negative.

[FIGURE 1 OMITTED]

A biopsy was performed, and histology of the specimen identified extensive noncaseating and coalescent granulomas that were made up of macrophages, giant cells, and lymphocytes, findings that are typical of sarcoidosis (figure 2). Special stains for organisms that cause granulomas, such as fungi and Mycobacterium tuberculosis, were negative. The underlying cartilage was normal. A chest x-ray showed bilateral hilar lymphadenopathy consistent with pulmonary sarcoidosis. The patient was referred to a respiratory physician for further management, and sarcoidosis was confirmed on transbronchial biopsy.

[FIGURE 2 OMITTED]

Discussion

The overall incidence of sarcoidosis has been reported to range from 1 to 40 cases per 100,000 population. (6) In most patients, the disease first manifests between the ages of 20 and 40 years. (7) More women are affected than men. Historically, Hutchinson is credited with the first description of this disease in 1875. (8) He named it Mortimer's malady after his patient, a Mrs. Mortimer, who had the unique skin findings. In 1899, Boeck described the skin manifestations in more detail, and he called the disease Boeck's sarcoidosis. (8) He used the root word sarcoid in the name because he thought it was a cutaneous form of sarcoma. The Danish ophthalmologist Heerfordt described the triad of uveitis, parotid enlargement, and cranial nerve paresis in 1909. (9) Schaumann described the pathologic findings in 1916. (10) Finally, there is a skin test for sarcoidosis that involves the use of a heat-killed suspension from the lymph nodes of known sarcoid patients. (11)

Sarcoidosis can manifest in many ways, and otolaryngologists must maintain an appropriate level of suspicion. By definition, sarcoidosis is a systemic disease. Hilar lymphadenopathy is present ill 90% of cases, and head and neck manifestations are found in 10 to 15% of cases. (12) Pulmonary symptoms often go unnoticed for some time and, as occurred in out- case, it is the head and neck manifestations that prompt the patient to seek medical attention. Findings on chest x-ray are used for staging irrespective of extrapulmonary involvement. A stage 1 designation is assigned to patients who have evidence of hilar adenopathy; these patients have an excellent prognosis, with a 60 to 80% chance of spontaneous resolution. (7) Stage II indicates hilar adenopathy with pulmonary infiltrates; stage 11 disease remits in 50 to 60% of patients. (7) Stage III indicates the presence of pulmonary infiltrates without adenopathy, and disease remits in fewer than 30% of patients. (7)

Otolaryngologic manifestations of sarcoidosis can be varied. The most common finding is cervical lymphadenopathy, and the second most common is skin lesions. (13) A variety of other conditions has been described as well, including lupus pernio, Hutchinson's plaque, and papular sarcoid. Sinonasal tract involvement has been reported in as many as 18% of cases. (14) Oral cavity manifestations of sarcoid are extremely rare. Parotid gland involvement is usually bilateral and nontender and has been reported in as many as 6% of cases. (15) Neurologic symptoms occur in only 5% of cases, but they are the most common reason for otolaryngologic consultation. (4) Facial nerve palsies are the most common cranial nerve deficit, but any cranial nerve can be affected. The paresis can be either fluctuating or progressive in nature. (7) Laryngeal sarcoid is present in 1 to 5% of all patients with sarcoidosis.(2) The supraglottic area is the most commonly affected site, but the subglottic region may also be involved. The true vocal folds are rarely affected. Localized pale edema, punctate submucosal nodules, and even a mass lesion have been described on laryngoscopy. (16)