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Revista Brasileira de Reumatologia
versão On-line ISSN 1809-4570
ZANON, Aline Biral et al. Primary hypertrophic osteoarthropathy: case report and literature review. Rev. Bras. Reumatol. [online]. 2009, vol.49, n.4, pp. 447-455. ISSN 1809-4570. http://dx.doi.org/10.1590/S0482-50042009000400010.
Primary hypertrophic osteoarthropathy is a rare syndrome, consisting of clubbed hands, fingers, and feet digits; enlarged extremities secondary to periarticular and bone proliferation; thickened facial skin; painful and swollen joints. The idiopathic form represents 3 to 5 per cent of all cases of hypertrophic osteoarthropaty. Genetic influence, abnormal fibroblasts activity, and changes of the peripherical blood flow appear to be significant on the pathogenesis. Clinical manifestations are variable: the term complete syndrome is used for the patient with pachydermia, coarsening of the face skin and scalp, periostitis, and cutis verticis gyrata); the incomplete form, when there is no sparing of the scalp; and the frusted form for pachydermia with minimal or absent periostitis. The authors describe a 39-year-old white man diagnosed with primary hypertrophic osteoarthropathy. We also report the clinical and radiological carateristics of this syndrome and terapeutical approach of pachydermoperiostosis.
Palavras-chave : hypertrophic osteoarthropathy; pachydermoperiostosis.