Red, Swollen, Tender Ear

The Diagnosis: Relapsing Polychondritis

Due to suspicion of relapsing polychondritis (RP), we also performed an audiometric evaluation, which demonstrated bilateral sensorineural hearing loss. Echocardiography highlighted mild to moderate mitralic and tricuspidal insufficiency without hemodynamic impairment (ejection fraction, 50%). Corticosteroid therapy was started (prednisone 0.5 mg/kg/d). After 7 days of treatment, inflammation was remarkably reduced, and the patient no longer reported pain.

Relapsing polychondritis is a rare noninfective condition characterized by focal inflammatory destruction of ear cartilage, followed by fibroblastic regeneration. It often is associated with ocular inflammation, including conjunctivitis, scleritis, and episcleritis; cochlear or vestibular lesions; and seronegative nonerosive inflammatory arthritis.¹ Clinical examination of the affected area shows swelling, redness, and tenderness of the ear, which could lead to a misdiagnosis of cellulitis. A typical and useful differentiating sign is the sparing of the noncartilaginous parts of the ear lobule. If not promptly diagnosed and treated, the destructive process can cause thinning of the cartilage, leading to deformities of the external ear.

The differential diagnosis includes erysipelas, which presents as a rapidly appearing inflammatory patch with sharply defined borders, accompanied by regional lymphadenopathy or skin streaking as well as fever. Sweet syndrome usually presents with tender erythematous or violaceous skin papules, plaques, or nodules, frequently with a pseudovesicular appearance; patients generally present with a classic fever and peripheral neutrophilia.² The localized cutaneous form of leishmaniasis usually appears with a papule that generally develops into an ulcerative nodular lesion. Our patient did not have a history of exposure to topical substances that could point to photocontact dermatitis.

Dion et al³ proposed 3 distinct clinical phenotypes of RP: (1) patients with concomitant myelodysplastic syndrome or other hematologic malignancy (<10% of patients), mostly older men with a poor prognosis; (2) patients with tracheobronchial involvement (approximately 25% of patients); and (3) patients who do not have hematologic or tracheobronchial involvement (approximately 65% of patients) with a good prognosis.

Two sets of diagnostic criteria have been proposed. The criteria from McAdam et al⁴ required the presence of 3 or more of the following clinical features: bilateral auricular chondritis, nonerosive seronegative inflammatory polyarthritis, nasal chondritis, ocular inflammation (eg, conjunctivitis, keratitis, scleritis/episcleritis, uveitis), respiratory tract chondritis (laryngeal and/or tracheal cartilages), and cochlear and/or vestibular dysfunction (eg, neurosensory hearing loss, tinnitus, vertigo). These criteria were modified by Damiani and Levine.⁵ According to the latter, all patients were required to have one of the following: at least 4 of the McAdam et al⁴ diagnostic criteria; 1 or more of the clinical findings included in the McAdam et al⁴ criteria with histologic features suggestive for RP; or chondritis at 2 or more separate anatomic locations with a response to glucocorticoids and/or dapsone.

No laboratory findings are specific for RP, and nonspecific indicators of inflammation--elevated erythrocyte sedimentation rate and C-reactive protein--often are present.
The cause of RP is unknown. Familial clustering has not been observed. Terao et al⁶ found that HLA-DRB1*1602, -DQB1*0502, and -B*6701, in linkage disequilibrium with each other, are associated with susceptibility to RP.

There is no universal consensus about treatment, but a course of steroids leads to the resolution of the acute phase. Maintenance treatment can include dapsone, azathioprine, methotrexate, cyclophosphamide, and cyclosporine.^7,8 Some studies have described the successful use of anti-tumor necrosis factor α inhibitors and rituximab.^9,10