Fibroblastic rheumatism.
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.
Marconi
I M L
IM
Department of Dermatology, Hospital das Clínicas, University of São Paulo, São Paulo, Brazil. isabelamarconi@gmail.com
Rivitti-Machado
M C
MC
Sotto
M N
MN
Nico
M M S
MM
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