A Rare Cause of Ear Pain in a Young Boy

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.

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• Figure 1 

  Hyperpigmentation of the left pinna due to chronic inflammation. The ear was tender to palpation.

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• 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.