Relapsing Polychondritis | Figure 3.37
A: Telangiectasias in CREST
(Courtesy of Dr. Paul Getz)
B: Relapsing polychondritis
(Reprint from Morgan MB,
Smoller BR, Somach SC.
Deadly Dermatologic Diseases.
New York, NY: Springer; 2007)
C: Lichen sclerosus
(Courtesy of Dr. Iris K.
Aronson) |
- Episodic inflammatory condition involving cartilaginous structures (ear) with suspected autoimmune origin
- Antibodies: anti-type II collagen (<50%), ± anti-matrilin-1 (cartilage extracellular matrix protein)
- Presents with episodes of painful, beefy red erythema and edema of cartilaginous portion of ears (earlobes spared); over time cartilage destroyed (‘cauliflower’ or floppy ears); nasal chondritis (saddle nose deformity), respiratory tract involvement (hoarseness), migratory arthralgia, ↓ hearing (deafness, tinnitus), ocular symptoms
- Histology: perichondrial inflammation with neutrophils, plasma cells or lymphocytes, cartilage degeneration (↓ basophilia, vacuolization of chondrocytes); advanced lesions with perichondrial fibrosis
- Treatment: oral corticosteroids, dapsone, other immunosuppressants (MTX, azathioprine, etc.)
- Associated with myelodysplastic syndrome, Behcet’s disease (MAGIC syndrome)
- Association: HLA-DR4
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