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Erosive Arthropathy with Osteolysis As a Typical Feature in Polyfibromatosis Syndrome: A Case Report and a Review of the Literature

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...

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Detalles Bibliográficos
Autores principales: Kim, Seong-Kyu, Kim, Hyung Joon, Lee, Young Hwan, Suh, Kyung Jin, Park, Sung-Hoon, Choe, Jung-Yoon
Formato: Texto
Lenguaje:English
Publicado: The Korean Academy of Medical Sciences 2009
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2672137/
https://www.ncbi.nlm.nih.gov/pubmed/19399279
http://dx.doi.org/10.3346/jkms.2009.24.2.326
Descripción
Sumario: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.