[Primary hypertrophic osteoarthropathy (pachydermoperiostosis). Report of two familial cases and literature review].
نویسندگان
چکیده
The primary hypertrophic osteoarthropathy (pachydermoperiostosis) is a hereditary disease characterized by skin thickening (pachydermia), finger clubbing, and proliferation of periosteum (periostitis) with subperiosteal new bone formation. We describe the cases of two brothers of 30 and 24 years, who consulted due to bone pain, arthralgia and oligoarthritis. Pachydermia, hyperhidrosis, seborrhea, digital clubbing, periostosis and non-inflammatory effusions of the knees. The first had been diagnosed with juvenile idiopathic arthritis at age 15, while the youngest also presented with a thoracic scoliosis, hypertrophic gastritis, iron deficiency anemia and glucose intolerance by pancreatic endocrine dysfunction. In both patients, symptoms were controlled satisfactorily with etoricoxib (90mg/day) and risedronate (35mg/week).
منابع مشابه
Primary hypertrophic osteoarthropathy: case report and literature review
Hypertrophic osteoarthropathy is a pathology characterized by digital clubbing of the hands and feet, extremity enlargement secondary to bone and periarticular tissue proliferation, joint pain and edema, bilateral eyelid ptosis, leonine face and skin thickness.1,2 It is classified as either primary (pachydermoperiostosis) or secondary. The primary form, or idiopathic, is considered rare, corres...
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Pachydermoperiostosis (PDP) is a rare disorder characterized by pachydermia, digital clubbing, periostitis, and an excess of affected males. It is the primary form of hypertrophic osteoarthropathy (HOA) and there are some rare associations of PDP with other disorders. Here we describe a patient with Crohn’s disease associated with PDP. A 26-year-old man, who was a known case of Crohn’s disease,...
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INTRODUCTION Pachydermoperiostosis or primary hypertrophic osteoathropathy is a rare genetic disease with autosomal transmission. This disorder, which affects both bones and skin, is characterized by the association of dermatologic changes (pachydermia or thickening of the skin) and rheumatologic manifestations (periostosis and finger clubbing). Here, we report a new observation of pachydermope...
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ورودعنوان ژورنال:
- Reumatologia clinica
دوره 5 6 شماره
صفحات -
تاریخ انتشار 2009