Primary pachydermoperiostosis
Terwindt, V.A.; Misere, J.F.; van Etten, R.J.
Netherlands Journal of Medicine 25(6): 167-174
1982
ISSN/ISBN: 0300-2977 PMID: 7121650 Document Number: 195857
Primary or idiopathic pachydermoperiostosis is a rare familial disease, characterized by hypertrophic changes of the skin and skeleton. A survey of the literature and the case histories of 2 brothers with this disease are presented. The skeletal abnormalities and differential diagnosis, especially from the secondary or acquired form of pachydermoperiostosis, are discussed in detail. The 2 brothers showed the type of periostosis which, according to numerous data in the literature, is the sole and distinguishing bone disorder of this disease. In the bone biopsy specimen from one of them, who showed rather extensive involvement of the skeleton, osteosclerosis and increased turnover of cancellous bone were demonstrable. These cancellous bone abnormalities are perhaps less rare than the literature seems to suggest.