Polyarthritis is a Rare Manifestation of Pachydermoperiostosis: A Case Report.

ABSTRACT

Pachydermoperiostosis or primary hypertrophic osteoarthropathy, also known as Touraine-Solente-Gole syndrome, is a rare process, frequently inherited. In its complete form it is characterized by pachydermia (thickening of the skin), skeletal changes (periostosis) and acropachia (digital clubbing). Diagnosis can be made considering the typical clinical features and the histological feature. We report a patient of 25-year old man presented with joint pain involving in multiple joints for last 7 years and progressive enlargement of his hands and feet with profuse sweating of palms and soles for last 4 years. Physical examination revealed thickened skin with excessive furrowing of his forehead, dropping of both eyelids, clubbing of all fingers, toes and enlargement of his hands and feet with pamoplantar hyperhidrosis. Laboratory investigation shows raised CRP, X-ray feet lateral view showed normal heel pad thickness, new bone formation and periosteal elevation in lower end of tibia and fibula and skin biopsy showed dense fibrocollagenous tissue in dermis and subcutis and mild acanthosis. With this scenario he was diagnosed as primary hypertrophic osteoarthropathy (Pachydermoperiostosis). After diagnosis he was treated with cholchicine (0.6mg) twice daily, naproxen (500mg) twice daily, and risedronate (150mg) monthly. After one year his skin texture became less thickened, joint pain improved, there was no further enlargement of acral part of fore arm. In this report we review the characteristic features of this syndrome. We highlight the importance of ruling out secondary forms of hypertrophic osteoarthropathy and of a close follow-up of these patients because of complications that might develop on long-term. Although no treatment was satisfactory, we wanted to emphasize that NSAIDs, Cholchicine and risedronate could be an effective treatment option.