Rheumatologic manifestations of pachydermoperiostosis and preliminary experience with bisphosphonates.

Abstract

Pachydermoperiostosis is manifested by finger clubbing, hypertrophic skin changes, and periosteal bone formation. We describe 5 cases revealed primarily by their rheumatologic manifestations. Also reported are preliminary experiences on the use of intravenous pamidronate as a treatment. This is a retrospective study including the analysis of clinical manifestations, laboratory results and morphological examinations gathered from patients' medical records. We evaluated efficacy of treatment with 1 mg/kg iv pamidronate in the 3 new cases. Before treatment with iv pamidronate, the patients' global assessment was poor (twice) and very poor (once). The physician's global assessment was poor in 3 patients. After treatment with iv pamidronate, 2 patients had significant improvement. Physician and patient global assessments were very good, good, and moderate. No side effects were observed. All biological variables were within normal ranges at 12 month followup visit. Pachydermoperiostosis must be recognized by the rheumatologist, since it can present symptomatically through articular manifestations. When conventional treatment modalities fail, iv pamidronate might be useful.