Pachydermoperiostosis

Abstract

Pachydermoperiostosis (primary or idiopathic hypertrophic osteoarthropathy) is a disorder characterized by the insidious development, usually during adolescence, of clubbing, cylindrical thickening of the legs and forearms involving soft tissues as well as bones; thickening and greasiness of the skin of the face, scalp, and forehead, with deep furrows, especially of the forehead and scalp (cutis verticis gyrata); and excessive sweating. 1,2 Radiologically, there is a symmetrical, irregular, subperiosteal ossification of the long bones, especially distally. The condition appears to be inherited in an autosomal fashion; there is marked variability in expressivity, with men, generally, being more severely affected. 3 In secondary clubbing (pulmonary osteoarthropathy), increased peripheral blood flow has been demonstrated repeatedly and has been thought to be induced by a vagal reflex mechanism, which is reversible. 4,5 Since clubbing without pulmonary lesions is characteristic of pachydermoperiostosis, peripheral circulatory studies in this condition and comparisons with pulmonary osteoarthropathy are