Relapsing Polychondritis: A Review and Guide for the Dermatologist

Abstract

Relapsing polychondritis, or RP, is a rare connective tissue disease characterized by relapsing-remitting destructive inflammation of the cartilaginous and other proteoglycan-rich structures in the body. Given the relatively low incidence of RP, a concise clinically relevant guide, focusing on the cutaneous manifestations of this serious disease, is lacking. In this review, we provide the dermatologist with an approach to diagnosing RP and a guide to its initial work-up, and management. We close with an overview of the currently available treatment modalities for RP.

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

“…This could be correlated with a case series where the anti‐matrilin 1 antibodies were presented in 69% of the cases of respiratory tract chondritis in patients with RPC; this increase in matrilin‐1 antibodies resulted from the cartilage destruction . In addition, some cases suggest that events such as mechanical, chemical, infectious aggressions, and cancer directed toward cartilaginous structures may precipitate the onset of RPC in genetically susceptible patients …”

“…13,[19][20][21][22] In addition, some cases suggest that events such as mechanical, chemical, infectious aggressions, and cancer directed toward cartilaginous structures may precipitate the onset of RPC in genetically susceptible patients. 12,13,17,[19][20][21][22][23] The cellular response appears to be mediated by cytokines invasive maneuvers with ventilatory support and extensive medication as in our case and other studies described. [1][2][3]25,26,28,29 Chest radiography may reveal tracheal narrowing, consolidations, or atelectasis that are induced by airway obstruction, pulmonary edema, and calcification of tracheal or laryngeal cartilage.…”

“…In all groups, the most frequent manifestations were purpura and nodules (41%), followed by urticarial papules (32%), phlebitis (27%), and livedo reticularis (23%). In global terms, cutaneous manifestations were more related to MDS and vasculitis . In our patient, skin manifestations were not found (See Table ) …”

“…In global terms, cutaneous manifestations were more related to MDS and vasculitis. 5,12,14,24,26,28,29,36 In our patient, skin manifestations were not found (See Table 2). 1-3,14,33-40 The histological findings help to guide the diagnosis in nonclassic cases.…”

“…Also, the treatment is not established, and there are no algorithms for the management of it, which causes confusion in the medical field. However, owing to the resistance to corticosteroids and therapeutic failure with conventional immunosuppressants, the advent of biological therapy represents a hopeful third line of management …”