Pachydermoperiostosis with insufficient valve function of deep vein

Abstract

Pachydermoperiostosis (PDP), or primary hypertrophic osteoarthropathy (PHO), is a rare hereditary disease mainly affecting skin and bones. The common clinical manifestations include digital clubbing, periostosis, and pachydermia. Patients often visit physicians because of thickened skin. However, the major concern of our patient was severe oedema of lower limb, although thickened facial skin also presented. Ascending phlebography revealed insufficient valve function of deep vein in lower limb. A reconstructive surgery was performed by enhancing the valve with tendon and by ligation of the great saphenous vein. Based on previous literatures, we hypothesize that the mechanism underlying the abnormalities in the valve of deep vein may be similar to that in skin and bones. We first report a PDP patient with a valve insufficiency treated with reconstructive surgery and we suggest that phlebography should be performed when the PDP patients present with severe pitting oedema in lower limbs.

Keywords:

• Pachydermoperiostosis
• insufficient valve function
• reconstructive surgery

Conflict of interest

None.

Funding

This case report received no specific grant from any funding agency in the public, commercial, or not for-profit sectors.