An unusual case of external ear inflammation caused by sarcoidosis.
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.
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.
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]
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)
The differential diagnosis of sarcoidosis can be exhaustive, and the diagnosis depends to a certain extent on the sites affected and the clinical signs and symptoms. Diagnosis requires a thorough physical examination, chest x-ray, serologic testing, and tissue evaluation to rule out other granulomatous diseases. Therefore, it is important that biopsies in cases of possible sarcoidosis be analyzed by fungal and mycobacterial cultures and stains. Noncaseating granulomas are the hallmark of sarcoidosis. Serologic testing can support the diagnosis of sarcoidosis. No single test is diagnostic, but the serum ACE level is the most useful test. Serum ACE levels are elevated in 60% of patients with sarcoidosis. (17) The enzyme is concentrated in the pulmonary vasculature and is also found in bronchopulmonary lavage fluid, serum, and cerebrospinal fluid. The ACE level is useful not so much for its diagnostic value as it is for its value as a measure of the "granuloma load" and as a marker of a patient's response to therapy.
Although the cause or trigger of sarcoidosis remains unknown, the pathogenesis of its many manifestations is becoming more fully understood. We do know that a specific agent or trigger stimulates local macrophages. The stimulated macrophages secrete chemotactic factors such as lymphokines, which then attract T-helper (T4) lymphocytes. The T4 cells in turn stimulate antibody production by B lymphocytes. Together the activated macrophages, B lymphocytes, and antibodies form granulomas in the affected tissue. (18) This overactive local immune response depletes the systemic immune system, thus creating an immunologically compromised state.
Many theories have been offered to explain what triggers this immune response. The theory that it is a transmissible agent, such as M tuberculosis, has received the most attention. Investigations reported by Bowman et al (19) in 1973 and later by Mitchell et al (20) in 1976 failed to isolate or identify any bacterial, fungal, or mycobacterial agents in human sarcoid granulomas. Others have argued that sarcoidosis represents a unique form of tuberculosis that is difficult to culture. More recent studies by Mitchell et al (21) and by Saboor et al (22) have renewed interest in this possibility. Mitchell's group detected ribosomal RNA specific to M tuberculosis in splenic tissue of sarcoid patients. (21) Saboor's group found Mycobacterium species DNA on polymerase chain reaction in the bronchopulmonary lavage fluid of 70% of sarcoid patients, compared with only 9% of controls. (22)
Before a treatment option for sarcoidosis is considered, patients would ideally be observed for all initial period during which no treatment is administered, because it is possible that the disease will resolve spontaneously. (23) However, when pulmonary manifestations are present, treatment is generally required. Treatment of extrapulmonary manifestations is required when deforming skin lesions are present and in cases of neurologic, ocular, major organ, or aerodigestive tract involvement. Oral steroids remain the cornerstone of treatment, but there is no set protocol. Patients are usually treated for at least 6 months initially. The ACE level or the erythrocyte sedimentation rate can be used to monitor the response to the medical regimen. Local steroids, either as topical ointments or as nasal sprays, can be of benefit in cases of skin or nasal involvement. The prognosis for patients who require treatment is good; more than 70% of such patients either improve or experience no further progression of their disease. (7) Those with progressive disease can experience a relentless course that can end in death secondary to either respiratory or cardiac failure.
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(2.) Weiss JA. Sarcoidosis in otolaryngology: Report of eleven cases: Evaluation of blind biopsy as a diagnostic aid. Laryngoscope 1960;70:1351-98.
(3.) Devine KD. Sarcoidosis and sarcoidosis of the larynx. Laryngoscope 1965;75:533-69.
(4.) Sharma OP, Sharma AM. Sarcoidosis of the nervous system. A clinical approach. Arch Intern Med 1991; 151:1317-21.
(5.) Mann RJ, Peaehcy RD. Sarcoidal tissue reaction another complication of ear piercing. Clin Exp Dermatol 1983:8:199-200.
(6.) Bresnitz EA, Strom BL. Epidemiology of sarcoidosis. Epidemiol Rev 1983;5:124-56.
(7.) Newman LS, Rose CS, Maier LA. Sareoidosis. N Engl J Mcd 1997;336:1224-34.
(8.) Hunter FT. Hutehinson-Boeck's disease (generalized sarcoidosis): Historical note and report of a case with apparent cure. N Engl J Med 1936;214:346.
(9.) Heerfordt CF. Uber cine "Febris Uveo-Parotidea Subchronica," an der Glandula Parotis und der Urea des Auges Lokalisiert und Jaufig mit Paresen Cerebrospinaler Nerven Kompliziert. Albrecht von Graefes Arch Ophthalmol 1909;70:254.
(10.) Schaumann J. Etude sur le lupus pemio et ses rapports avec les sarcoides et la tuberculose. Ann Dermatol Syphil 1916;6:357.
(11.) Munro CS, Mitchell DN, Poultcr LW, Cole PJ. Early cellular responses to intradermal injection of Kveim suspension in normal subjects and those with sarcoidosis. J Clin Pathol 1986;39: 176-82.
(12.) McCaffrey TV. McDonald TJ. Sarcoidosis of the nose and paranasal sinuses. Laryngoscope 1983;93:1281 4.
(13.) Dash GI, Kimmelman CP. Head and neck manifestations of sarcoidosis, Laryngoscope 1988;98:50-3.
(14.) James DG. Lupus pernio. Lupus 1992;1:129-31.
(15.) Maier H. Bihl H, Born IA, Adler D. [Sarcoidosis (Boeck disease) of the parotid gland]. Laryngol Rhinol Otol (Stuttg) 1985;64: 537-41.
(16.) Benjamin B, Dalton C, Croxson G. Laryngoscopic diagnosis of laryngeal sarcoid. Ann Otol Rhinol Laryngol 1995;104:529-31.
(17.) Dietemann-Molard A, Pelletier A, Pauli G, et al. [Practical value of the assay of serum angiotensin converting enzyme activity in sarcoidosis]. Rev Pneumol Clin 1984;40:121-5.
(18.) Hunninghake GW, Crystal RG. Pulmonary sarcoidosis: A disorder mediated by excess helper T lymphocyte activity at sites of disease activity. N Engl J Med 1981;305:429-34.
(19.) Bowman BU, Koehler RM, Kuhina G. On the isolation of infectious agents from granulomas of patients with sarcoid. Am Rev Respir Dis 1973;107:467-8.
(20.) Mitchell DN, Rees R J, Goswami KK. Transmissible agents from human sarcoid and Crohn's disease tissues. Lancet 1976; 2(7989):761 5.
(21.) Mitchell IC, Turk JL. Mitchell DN. Detection of mycobacterial rRNA in sarcoidosis with liquid phase hybridisation. Lancet 1992;339:1015-17.
(22.) Saboor SA, Johnson NM, McFadden J. Detection of mycohacterial DNA in sarcoidosis and tuberculosis with polymerase chain reaction, Lancet 1992;339:1012-15.
(23.) Gibson GJ, Prescott R J, Muers MF, et at. British Thoracic Society Sarcoidosis study: Effects of long term corticosteroid treatment. Thorax 1996;51:238-47.
From the Department of Otolaryngology--Head and Neck Surgery (Dr. Lang, Dr. El Zaruk, Dr. Colreavy, Dr. Rowley, and Dr. Timon) and the Department of Pathology (Dr. Kennedy), Royal Victoria Eye and Ear Hospital, Dublin, Ireland.
Reprint requests: Dr. E. E. Lang, 36 Raheny Park, Raheny, Dublin 5, Ireland. Phone: 353 1 831-2588; e-mail: email@example.com
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|Title Annotation:||Original Article|
|Publication:||Ear, Nose and Throat Journal|
|Date:||Dec 1, 2003|
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