Korean J Dermatol.  2010 May;48(5):445-448.

A Case of Pachydermoperiostosis

Affiliations
  • 1Department of Dermatology, CHA Bundang Medical Center, CHA University College of Medicine, Seongnam, Korea. msyoon@unitel.co.kr
  • 2Department of Pathology, CHA Bundang Medical Center, CHA University College of Medicine, Seongnam, Korea.

Abstract

Pachydermoperiostosis is a very rare genodermatosis that is characterized by pachydermia, digital clubbing and periostosis. It usually occurs during adolescence and the disease is inherited in an autosomal dominant manner. Although the pathogenesis of this disease is still a matter of debate, it is associated with abnormal proliferation of fibroblasts, osteoblasts and endothelial cells and altered proteoglycan synthesis. We report here on a case of pachydermoperiostosis in a 21-year-old man who was diagnosed by the clinical features, skin biopsy and radiological examinations.

Keyword

Pachydermoperiostosis

MeSH Terms

Adolescent
Biopsy
Endothelial Cells
Fibroblasts
Humans
Osteoarthropathy, Primary Hypertrophic
Osteoblasts
Proteoglycans
Skin
Young Adult
Proteoglycans
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