Background: The diagnosis of relapsing polychondritis is difficult as various manifestations may be encountered aside the characteristic episodes of recurrent chondritis.

Methods: From the retrospective analysis of the medical charts of patients presenting with relapsing polychondritis seen at Nîmes hospital between 1995 to 2006, four were selected for their original extra-cartilaginous manifestations. Case 1: relapsing polychondritis was diagnosed at the time of a thromboembolic event associated with a right uveitis, left temporomandibular arthritis and bilateral sensorineural deafness. Case 2: relapsing polychondritis occurred in a patient with history of bilateral sensorineural deafness with punctuated keratitis followed by a relapsing cutaneous leucocytoclastic vasculitis. Case 3: relapsing polychondritis associated with recurrent thromboembolic disease and a Sweet's syndrome and case 4: relapsing polychondritis presenting with a febrile erythema.

Conclusions: The reported observations highlight the difficulty of the initial diagnosis of relapsing chondritis and the variety of the extra-chondritis manifestations that could be observed in this disease.