中文摘要 |
Pachydermoperiostosis (PDP) orprimary hypertrophic osteoarthropathy,is a rare hereditary disorder thatmainly appears as in autosomaldominant pattern with variablepenetration. A 19-year-old manpresented with painful swellingand stiffness of the knee andprogressive enlargement of bothhands and feet for the past 4 years.He had no other pertinent medicalor familial history. The patient hadnormal blood pressure, oily skin inconjunction with hyperhidrosis, acneand folliculitis on the trunk (Figure1). Clubbing of the fingers wasobserved(Figure 2). Laboratory testsresults were normal for levels of insulin-like growth factor-1, thyroid function and growth hormone.A peripheral blood test revealed a white blood count of 10,200/μL, hemoglobin count of 11.2 g/dL,and platelet count of 227,000/μL. The erythrocyte sedimentation rate and C-reactive protein levelwere elevated at 56 mm/h and 1.8 mg/dL (<5), respectively. Negative results were obtained forrheumatoid factor, anti-cyclic citrullinated protein antibodies, HLA-B27 and antinuclear antibody.X-rays revealed hypertrophic change with periosteal thickening of the long bones and narrowing ofthe joint space over the medial part of bilateral knees (Figure 3).The presence of clubbing and radiographic evidence of periostosis of the tubular bones withoutunderlying diseases led to the diagnosis of pachydermoperiostosis in this patient.The clues fordifferential diagnosis are digital clubbing and periostosis, which are not features of acromegaly. |