title: Cutaneous manifestations of patients with relapsing polychondritis: an association with extracutaneous complications.
abstract: The aim of this study was to determine the prognostic characteristics of patients with relapsing polychondritis (RP) accompanying cutaneous manifestations in Japan. We analyzed a cohort of 239 patients with RP in view of cutaneous and extracutaneous complications. Thirty three cases (14%) developed cutaneous manifestations and 23 cases had both cutaneous and extracutaneous manifestations. Five RP patients developed myelodysplastic syndrome (MDS) and all of the five patients had cutaneous manifestations, including Sweet's syndrome. Only one patient died of MDS among the five patients, suggesting rather better prognosis as compared with ordinary MDS. Five RP patients developed Behcet's disease and all the five patients had cutaneous manifestations. Death rate of the RP patients with cutaneous manifestations (15%) was slightly higher than that of whole Japanese RP patient cohort (9.2%). RP patients with cutaneous manifestations had a slightly higher death rate, than those without cutaneous manifestations. MDS of RP patients had a rather better prognostic impact in Japan. Further studies are needed to elucidate the pathophysiology of RP, which brings about development of extracutaneous manifestations, especially MDS and Behcet's disease.
authors.affiliation: Institute of Medical Science and Departments of Immunology and Medicine, St. Marianna University School of Medicine, Kawasaki, 216-8511, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Shimizu
authors.affiliation: Rheumatic Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, Tokyo, 193-0998, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Oka
authors.affiliation: Institute of Medical Science and Departments of Immunology and Medicine, St. Marianna University School of Medicine, Kawasaki, 216-8511, Japan.
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Yamano
authors.affiliation: Institute of Medical Science and Departments of Immunology and Medicine, St. Marianna University School of Medicine, Kawasaki, 216-8511, Japan.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Yudoh
authors.affiliation: Institute of Medical Science and Departments of Immunology and Medicine, St. Marianna University School of Medicine, Kawasaki, 216-8511, Japan. n3suzuki@marianna-u.ac.jp.
authors.forename: Noboru
authors.initials: N
authors.lastname: Suzuki
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000209:etiology / Q000473:pathology*; D011081:Polychondritis, Relapsing / Q000150:complications / Q000473:pathology*; D011379:Prognosis; D012867:Skin / Q000473:pathology*; D016463:Sweet Syndrome / Q000209:etiology / Q000473:pathology*; D055815:Young Adult
keywords: Behcet’s disease; Cutaneous manifestations; Epidemiology; Myelodysplastic syndrome; Relapsing polychondritis