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J Korean Med Sci. 2009 Apr;24(2):326-9. doi: 10.3346/jkms.2009.24.2.326. Epub 2009 Apr 20.

Erosive arthropathy with osteolysis as a typical feature in polyfibromatosis syndrome: a case report and a review of the literature.

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  • 1Department of Internal Medicine, Catholic University of Daegu School of Medicine, Daegu, Korea.

Abstract

Polyfibromatosis syndrome is a rare disease entity that is characterized by various clinical features such as palmar, plantar, and penile fibromatoses, keloid formations of the skin, and erosive arthropathy. Its precise pathophysiology or etiology remains unclear. In addition to distinctive diverse skin manifestations, patients with polyfibromatosis have been previously reported to show erosive arthropathy with significant limitation of movement at affected joints. However, the presence of erosive polyarthropathy in polyfibromatosis has not emphasized in previous cases. Here, we report a case of polyfibromatosis syndrome combined with painless massive structural destruction of hand and foot joints, and review the characteristics of erosive arthropathy in previous cases.

KEYWORDS:

Erosive Arthropathy; Magnetic Resonance Imaging; Polyfibromatosis

PMID:
19399279
PMCID:
PMC2672137
DOI:
10.3346/jkms.2009.24.2.326
[PubMed - indexed for MEDLINE]
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