Orbital mucosa-associated lymphoid tissue (MALT)-type lymphoma in a patient with relapsing polychondritis

Relapsing polychondritis is characterized by recurrent inflammation of the cartilaginous tissues of the ears, nose, peripheral joints, and the tracheo bronchial tree. The eye is also a frequent target organ in relapsing polych ondritis, and proptosis is a well-recognized manifestation of eye involveme nt. Similar to other rheumatologic diseases, an association of relapsing po lychondritis with malignancy has been reported. We describe a patient with relapsing polychondritis who presented with exophthalmos. When treatment di rected toward control of her underlying disease was only partially effectiv e, further investigation revealed that she had an orbital mucosa-associated lymphoid tissue (MALT)-type B cell lymphoma. We hypothesize that the lymph oma resulted from malignant transformation of the relapsing polychondritis- induced inflammatory pseudotumor and emphasize that neoplastic disease shou ld be considered in the differential diagnosis in patients with relapsing p olychondritis presenting with exophthalmos.