A case of relapsing polychondritis and pyoderma gangrenosum in a patient with Down's syndrome / 대한내과학회지

ABSTRACT

Relapsing polychondritis (RP) is a rare multisystem disorder of unknown etiology that affects cartilaginous tissues, such as the auricular, nasal, and laryngotracheal cartilages. It may be accompanied by a wide spectrum of skin lesions, including erythema nodosum, erythema multiforme, and panniculitis. Pyoderma gangrenosum is a rare chronic cutaneous disease that usually presents as a painful nodule or pustule and progressively forms an enlarging ulcer. It may be associated with inflammatory bowel disease, rheumatoid arthritis, systemic lupus erythromatosus, leukemia, and myeloproliferative disorders. Pyoderma gangrenosum is rarely associated with RP. RP and pyoderma gangrenosum have been reported in a patient with myelodysplastic syndrome (MDS), and the appearance of skin lesions in MDS may herald its progression to acute myeloid leukemia. Here, we report the first case of RP coexisting with pyoderma gangrenosum in a patient with Down's syndrome.