Marconi I M L, Rivitti-Machado M C, Sotto M N, Nico M M S
Department of Dermatology, Hospital das Clínicas, University of São Paulo, São Paulo, Brazil.
Clin Exp Dermatol. 2009 Jan;34(1):29-32. doi: 10.1111/j.1365-2230.2008.02748.x.
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.