title: Fibroblastic rheumatism.
abstract: Fibroblastic rheumatism (FR) was first described in 1980 by Chaouat et al., and there have been few cases reported to date. The cause remains unknown. We report the first Latin-American patient with FR, to our knowledge, who is also the patient with the most striking dermatological features described in the literature. The diagnosis was based on the presence of a number of typical features. Clinically, the patient presented skin nodules and polyarthropathy with flexion contractures of the fingers. The histological findings compressed fibroblastic proliferation, thickened collagen fibres, dermal fibrosis and a decreased number of elastic fibres. Immunoreactivity for beta-catenin, alpha-smooth muscle actin and the monoclonal antibody HHF-35 showed myofibroblastic differentiation. Treatment with prednisone slightly reduced the number of nodules but did not improve the rheumatological symptoms. This condition has shown a poor response to many treatments proposed by previous authors. Further study will be necessary to identify effective treatment.
authors.affiliation: Department of Dermatology, Hospital das Clínicas, University of São Paulo, São Paulo, Brazil. isabelamarconi@gmail.com
authors.forename: I M L
authors.initials: IM
authors.lastname: Marconi
authors.forename: M C
authors.initials: MC
authors.lastname: Rivitti-Machado
authors.forename: M N
authors.initials: MN
authors.lastname: Sotto
authors.forename: M M S
authors.initials: MM
authors.lastname: Nico
mesh_terms: D000328:Adult; D001938:Brazil; D004547:Elastic Tissue / Q000473:pathology*; D006801:Humans; D008297:Male; D012216:Rheumatic Diseases / Q000473:pathology*; D012595:Scleroderma, Systemic / Q000473:pathology; D012867:Skin / Q000473:pathology*; D013577:Syndrome