Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 3 1 2018 07 24 2 6 EN Shima Asadi-Komeleh Department of Rheumatology, School of Medicine AND Vali-Asr Hospital, Tehran University of Medical Sciences, Tehran, Iran. Abdolrahman Rostamian Department of Rheumatology, School of Medicine AND Vali-Asr Hospital AND Center for Research on Occupational Disease, Tehran University of Medical Sciences, Tehran, Iran. Fatemeh Shahbazi Department of Biology, Payame Noor University, Tehran, Iran. Shafieh Movassagi Department of Rheumatology, School of Medicine AND Vali-Asr Hospital, Tehran University of Medical Sciences, Tehran, Iran. Parviz Soofivand Department of Rheumatology, School of Medicine AND Vali-Asr Hospital, Tehran University of Medical Sciences, Tehran, Iran. 2017 10 26 2018 03 17 The primary hypertrophic osteoarthropathy (PHOA or pachydermoperiostosis) is a rare (5% of total HOA) hereditary disease. One study described that the prevalence of PHOA is 0.16%. PHOA characterized by skin thickening (pachydermia), finger clubbing, and proliferation of periosteum (periostitis) with subperiosteal new bone formation and enlarged extremities secondary to periarticular and bone proliferation. Clinical manifestations are variable; the term complete syndrome is used for the patient with pachydermia, coarsening of the face skin and scalp, periostitis, and cutis verticis gyrata; the incomplete form is used when there is no sparing of the scalp; and the frusted form is used for pachydermia with minimal or absent periostitis. We describe a 29-year-old white man with PHOA, and clinical and radiological characteristics of this syndrome, as well as therapeutic approach of PHOA. https://crcp.tums.ac.ir/index.php/crcp/article/view/145 https://crcp.tums.ac.ir/index.php/crcp/article/download/145/97