A Case of Pachydermoperiostosis.
- Author:
Tae Yoon KIM
1
;
Se Rim CHOI
;
Tae Heon KIM
;
Dong Hyun KIM
;
Moon Soo YOON
Author Information
1. Department of Dermatology, CHA Bundang Medical Center, CHA University College of Medicine, Seongnam, Korea. msyoon@unitel.co.kr
- Publication Type:Case Report
- Keywords:
Pachydermoperiostosis
- MeSH:
Adolescent;
Biopsy;
Endothelial Cells;
Fibroblasts;
Humans;
Osteoarthropathy, Primary Hypertrophic;
Osteoblasts;
Proteoglycans;
Skin;
Young Adult
- From:Korean Journal of Dermatology
2010;48(5):445-448
- CountryRepublic of Korea
- Language:Korean
-
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.