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Dermatologic manifestations of relapsing polychondritis

Dermatologic manifestations of relapsing polychondritis
Dermatologic manifestation Relapsing polychondritis (no associated disease)
(n = 127)
Relapsing polychondritis with myelodysplastic syndrome (MDS)
(n = 22)
Relapsing polychondritis with (non-MDS) associated disease*
(n = 51)
Number (percent) Number (percent) Number (percent)
Total with skin manifestation 45 (35) 20 (91) ––
Oral aphthosis only 14 (11) 2 (9) 4 (8)
Complex aphthosis 7 (6) 2 (9) 3 (6)
White oral lesion 0 (0) 1 (5) 0 (0)
Purpura 13 (10) 9 (41) 4 (8)
Livedo reticularis 7 (6) 5 (23) 2 (4)
Urticarial papules 5 (4) 7 (32) 4 (8)
Bluish-red papules 5 (4) 5 (23) 0 (0)
Erythema elevatum diutinum 1 (1) 1 (5) 0 (0)
Nodules on the limbs 19 (15) 9 (41) 7 (14)
Superficial phlebitis 8 (6) 6 (27) 1 (2)
Sterile pustules 9 (7) 4 (18) 0 (0)
Ulcerations on the limbs 6 (5) 3 (14) 3 (6)
Distal necrosis 4 (3) 1 (5) 2 (4)
* Some manifestations may be related to either relapsing polychondritis or the associated disease. Associated diseases include systemic lupus erythematosus, rheumatoid arthritis, mixed connective tissue disease, dermatomyositis, granulomatosus with polyangiitis (Wegener's), autoimmune thyroid disease, spondyloarthritis, multiple myeloma, inflammatory bowel disease, and aseptic abscesses.
¶ Total number of patients with any manifestation not provided.
Original data from: Frances C, El Rassi R, Laporte JL, et al. Dermatologic manifestations of relapsing polychondritis: A study of 200 cases at a single center. Medicine 2001; 80:173.
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