Summary
Pachydermoperiostosis (PDP) is a rare hereditary disorder that is characterized by digital clubbing and subperiosteal new bone formation associated with pain, polyarthritis, cutis verticis gyrata, seborrhea, hyperhidrosis. The precise incidence of the disease is unknown. PDP is often familial and occurs predominantly in men. Three forms of PDP are described: complete, incomplete and fruste form. PDP is manifested mainly by dermatological (pachydermia, thickening and furrowing of the facial feature, digital clubbing, cutis verticis gyrata, seborrhea, oedema, hyperhidrosis) and rheumatological symptoms (joint effusion, arthritis, acro-osteolysis, periosteal ossification). It is believed to be inherited in an autosomal dominant pattern with variable penetrance; autosomal recessive forms have also been reported. Rheumatologiac symptoms can be improved by nonsteroidal anti-inflammatory drugs or corticosteroids or colchicine. Clinical improvement of the dermatological symptoms was achieved by retinoids. Plastic surgery may be helpful for complications on the face. Finger clubbing surgical reduction has been tried with success. *Authors: Dr M. Auger and Prof. Stavrianeas (April 2004)*.
