PACHYDERMOPERIOSTOSIS, or primary hypertrophic osteoarthropathy, is a rare disorder of bone and connective tissue growth. The disease is characterized by clubbing of the digits, thickening of the skin and subcutaneous tissue, and progressive enlargement of the bones of the extremities. Involvement of the face and scalp produces coarsening of the features, accentuated wrinkling, and leads eventually to a unique facial and skeletal appearance. The hereditary nature of the disease is suggested by the occurrence of some degree of involvement in siblings, parents, and grandparents of about 50% of the reported cases.1 Pachydermoperiostosis should be distinguished from the more familiar secondary hypertrophic osteoarthropathy which occurs in association with suppurative or neoplastic lung disease. Both forms of the disorder tend to produce identical physical, radiologic, and pathologic abnormalities which can be differentiated only by the setting in which they occur.
The purpose of this report is to describe the clinical features