Relapsing polychondritis is a rare multisystem autoimmune disease.
Different factors are implicated in the pathogenesis, including a genetic susceptibility, immunization against cartilaginous structures, and modification of cytokine and chemokine signatures.
More than 30% of patients have an associated disease, mainly of autoimmune or hematologic origin.
Recurrent episodes of chondritis lead to progressive destruction of cartilaginous structures.
Other proteoglycan-rich structures, such as eyes, blood vessels, or inner ear, are also affected.
Dermatologic manifestations occur frequently, especially in association with myelodysplasia. They are nonspecific and resemble those observed in Behçet disease and inflammatory bowel diseases.
Relapsing polychondritis (RP) is a rare inflammatory disease with an estimated prevalence of 4.5 per million people.1 The incidence of RP has been estimated to be 0.71/million/year in the United Kingdom, being slightly higher in women than men (0.76 vs 0.66). The mean age (range) at diagnosis in men was 55 years (range: 17-81 years) and in women 51 years (range: 11-79 years).2 Development of the disease may occur in young children and in the elderly. Although most cases have been reported in whites, there is no evidence supporting the role of ethnic or geographical factors.
Disease onset is usually sudden with characteristic chondritis, and/or, less frequently, arthritis or ocular inflammation. Nonspecific initial symptoms, such as fever or weight loss, are rare.
Attacks of chondritis usually occur in a relapsing–remitting pattern. Inflammatory episodes generally last a few days or weeks and may subside spontaneously or upon treatment; recurrences after weeks or months occur and subsequently result in cartilage destruction.3 Auricular chondritis is the most frequent occurrence (85% of cases), causing pain, redness, and swelling of the cartilaginous portion of the pinna, sparing the noncartilaginous lobe (Fig. 69-1). Biopsy of the auricular cartilage is unnecessary for diagnosis. After several attacks, the pinna may become soft and sloppy with a cauliflower aspect (Fig. 69-2); sometimes it is stiff from calcifications. Nasal chondritis (65% of cases) is less inflammatory, presenting with nasal pain, stuffiness, rhinorrhea, and sometimes epistaxis. The characteristic saddle-nose deformity (Fig. 69-3) may appear secondly or without previous inflammatory episodes.
Relapsing polychondritis. Painful inflammation of the cartilaginous portion of ear.
Relapsing polychondritis. Soft and sloppy ear with a “cauliflower” aspect.
Relapsing polychondritis. Saddle-nose deformity.
Other dermatologic manifestations are sometimes the presenting feature of RP (12% of cases), noticed subsequently in more than one-third of patients of a large series.4 The other dermatologic manifestations are nonspecific and include nodules on the limbs ...