A Rare Cause of Ear Pain in a Young Boy
Article Outline
A 7-year-old boy presented with a 3-month history of persistent left auricular swelling and pain. He had been previously treated with several rounds of antibiotics with minimal improvement. Subsequently, he developed swelling and pain in his third, fourth, and fifth fingers. Examination revealed a darkly pigmented patch of skin on the left pinna that was tender to palpation (Figure 1). Flexure deformities and periarticular swelling of the proximal interphalangeal joints of the third, fourth, and fifth digits were noted. A saddle nose deformity was also observed. Biopsy analysis of the auricular lesion revealed perichondritis and chondritis with eroded, focally necrotic cartilage (Figure 2). Collectively, these findings led to a diagnosis of relapsing polychondritis.
Figure 1
Hyperpigmentation of the left pinna due to chronic inflammation. The ear was tender to palpation.
Figure 2
A section of the cartilage showing perichondritis and chondritis, with the inflammatory infiltrate mainly lymphohistiocytic in nature and with a band-like distribution in the perichondrium. Focal chondrocyte necrosis and erosion of the cartilage are also evident. Immunofluorescence studies showed focal weak deposition of C3 (+2) in some peripheral chondrocytes, but those were considered nonspecific. There was no deposition of IgG, IgA, or IgM. Special stains with for acid-fast bacilli and fungal organisms revealed no organisms.
Relapsing polychondritis is a rare systemic inflammatory disease that targets the structural and functional integrity of cartilaginous structures. Less than 10% of reported cases occur in children and adolescents.1 Auricular chondritis is the most frequent clinical feature. Other manifestations include nonerosive inflammatory polyarthritis, nasal chondritis, scleritis, respiratory tract laryngeal and/or tracheal inflammation, audiovestibular abnormalities, and cardiovascular involvement, most commonly aortic insufficiency. Laboratory findings were nonspecific. Although the pathogenesis is unknown, antibodies to type II collagen are present in 20% of patients.2 Rheumatologic diseases and myelodysplastic syndromes are seen in conjunction with relapsing polychondritis in one-third of cases.3 Treatment consists of high-dose glucocorticoids, with the addition of steroid-sparing immunosuppression in cases of multiorgan involvement.
Prognosis is worse in younger patients and those with aggressive disease; in those cases, the reported mortality rate ranges from 28% to 50%.4 Despite aggressive immunosuppression with glucocorticoids and mycophenolate mofetil, our patient continued to decline and developed respiratory stridor within 7 months of diagnosis. Evaluation revealed posterior tracheal collapse above the carina and dynamic collapse on expiratory fluoroscopic images. Given the extent of tracheal involvement, tracheostomy could not be performed, and the patient ultimately succumbed to respiratory failure.
References
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PII: S0022-3476(11)00825-0
doi:10.1016/j.jpeds.2011.08.030
© 2012 Mosby, Inc. All rights reserved.
