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.
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- Relapsing polychondritis (RP) is a rare autoimmune disorder that causes recurring inflammation in cartilaginous tissues, affecting areas like the ears, nose, and joints.
- A 68-year-old woman without prior health issues showed symptoms like hoarseness, polyarthritis, and nasal/ear involvement, leading to her RP diagnosis using the Modified McAdam criteria.
- She was treated with intravenous cyclophosphamide and oral prednisolone, which improved her condition, while osteoporosis was treated with zoledronic acid, highlighting RP's role in patient assessments involving similar symptoms.
VEXAS syndrome initially presenting as relapsing polychondritis and progressing into neutrophilic dermatosis with systemic symptoms over a 5-year period.
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Department of Dermatology, International University of Health and Welfare, Chiba, Japan.
[VEXAS-like auto inflammatory syndrome: 2 cases].
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Introduction: VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic), recently described, due to a somatic mutation of the UBA1 gene and often associated with hemopathy, is characterized by systemic symptoms close to those described in Still's disease or relapsing polychondritis. There are also patients with hemopathy, presenting inflammatory symptoms reminiscent of those of VEXAS syndrome but without mutation of the UBA1 gene.
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Relapsing polychondritis is rare and affects non-synovial fibrocartilage. Currently, there is a paucity of treatment algorithms, especially for those with refractory disease. A middle-aged man presented with polychondritis affecting the nose, ears, joints, and larynx.
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