Relapsing polychondritis, an underestimated dermatological urgency: case report and literature review

Int J Dermatol. 2017 Dec;56(12):1379-1386. doi: 10.1111/ijd.13755. Epub 2017 Oct 9.

Abstract

Background: Relapsing polychondritis is an autoimmune multisystemic disease with primary chondral involvement. Its high mortality and morbidity make it a real clinical challenge.

Case description: A 32-year-old woman with a history of relapsing polychondritis, refractory to multiple treatments, with multisystem compromise, imminent risk of death due to severe tracheobronchial damage and difficult ventilatory support, and successful treatment with infliximab.

Discussion and evaluation: Several treatments have been described in the literature, such as nonsteroidal anti-inflammatory drugs, corticosteroids, dapsone, azathioprine, cyclosporine, cyclophosphamide, and methotrexate. However, the cases refractory to conventional therapy may lead to chronicity, irreversibility, and death. As a result, a third-line therapy could improve the prognosis of these patients.

Conclusions: Biological therapy is a good option for disease control and quality of life improvement. In addition, the physician should consider these treatments to avoid the chronicity and risk of death of these patients.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Dermatologic Agents / therapeutic use*
  • Female
  • Humans
  • Infliximab / therapeutic use*
  • Polychondritis, Relapsing / complications
  • Polychondritis, Relapsing / diagnosis
  • Polychondritis, Relapsing / drug therapy*
  • Salvage Therapy

Substances

  • Dermatologic Agents
  • Infliximab