Relapsing poly(peri)chondritis diagnosed by biopsy during inflammatory free interval: destructive polychondritis versus fibrosing perichondritis.

ABSTRACT

Relapsing poly(peri)chondritis (RP) is a connective tissue disorder characterized by recurrent inflammatory episodes of cartilaginous structures and the involvement of special sense organs. The diagnostic criteria of McAdam 1976 include at least three of the following criteria a) bilateral auricular chondritis, b) nonerosive sero-negative inflammatory polyarthritis, c) nasal chondritis, d) ocular inflammation, e) respiratory tract chondritis, f) audiovestibular chondritis. A cartilage biopsy according to these criteria is not mandatory. Nevertheless, unclear cases still remain as there is a broad spectrum of differential diagnosis. In these individuals it is important to obtain a biopsy from the affected cartilage. Although up to 89% develop auricular inflammation, only few electron microscope studies are performed on cartilage specimens. The purpose of this study is to report on a patient with a history of recurrent swelling of both ears, where the diagnosis could only be established by ear biopsy which was studied by light and electron microscopy. Differential diagnosis is discussed and a review of the literature is given.