Primary Hypertrophic Osteoarthropathy: A Case Report

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.