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The hematologic pheno- type was associated with death, the respiratory phenotype was associated with infections and admission to the intensive care unit, and the mild phenotype was associated with the absence of severe complications. This report was of great interest to us, because we also encountered a lethal case of RP complicated by myelodysplastic syndrome (MDS) and Sweet syndrome. The clin- ical course of this patient was quite unusual, suggesting that RP complicated by these diseases is a distinct syndrome, and develop- ment of specific therapeutic strategies is needed. We had been treating 14 RP patients at our institution (57.1% male, mean 6 SD age 53.36 6 16.51 years, mean 6 SD follow-up time 5.67 6 4.2 years). The affected cartilages were auricular (85.7%), nasal (14.3%), and tracheal (7.1%). In 57.1% of the patients, extracartilage symptoms developed, including encephalitis and meningitis, scleritis, uveitis, hypothyroidism, rheumatoid arthritis, Behc ¸et’s syndrome, IgA nephropathy, MDS, and Sweet syndrome. Patients were treated with cortico- steroids (mean 6 SD prednisolone dosage 42.3 6 20.9 mg/day), but most of the patients who were treated solely with steroids experienced a relapse (relapse rate 71.4%), and additional immu- nosuppressants (methotrexate, cyclosporine, or cyclophospha- mide) were needed. However, their survival was excellent except for a case that was complicated by MDS and Sweet syndrome. The patient, a 56-year-old man, developed arthritis, erup- tions, and swelling of the right auricular cartilage. Based on the symptoms together with the histologic findings (Figure 1A), he was diagnosed as having RP. One month later, erythema and papules appeared on his face, and he was diagnosed as having Sweet syndrome (Figure 1B). The next year, he developed uveitis and MDS (refractory anemia) (Figure 1C). He sometimes experi- enced disease flare, and it was difficult to reduce the dose of prednisolone below 15 mg even though other immunosuppressive drugs (methotrexate, cyclosporine, mizorivine, or azathioprine) were added. Two years after diagnosis, he was admitted to our hospi- tal to control his disease. He was treated with pulsed methylpred- Figure 1. Histologic findings in a patient with relapsing polychondri- tis and myelodysplastic syndrome. A, Biopsy specimen from the auri- cle, showing degeneration of the cartilage (arrowheads) and infiltration of inflammatory cells (arrow). Hematoxylin and eosin (H&E) stained. Bar 5 200 mm. B, Biopsy specimen from the skin, showing massive infiltration of neutrophils into the dermis and sub- cutaneous tissue, with leukocytoclastic vasculitis. H&E stained. Bar 5 50 mm. C, Bone marrow aspirate, showing a multinucleated erythroblast and hypogranular neutrophils with a pseudo2Pelger- Hu€ et nucleus. D, Computed tomography image, showing multiple small nodules in the lungs (top) and a mass in the left adrenal gland (bottom). E, Histologic assessment of a nodule in the left adrenal gland, showing viable cells with irregular and hyperchromatic nuclei. These cells were immunopositive for CD20 and CD79a, and positive for Epstein-Barr virus2encoded small RNA in situ hybridization (EBER-ISH). H&E stained. Bar 5 50 mm. 682 LETTERS