J Rheum Dis.  2013 Aug;20(4):247-250. 10.4078/jrd.2013.20.4.247.

A Case of Acroosteolysis in Primary Hypertrophic Osteoarthropathy

• Lee HJ
• Kim HJ
• Hwang CG
• Choi JH
• Kang EJ

• KMID: 2297540
• DOI: http://doi.org/10.4078/jrd.2013.20.4.247

Abstract

Primary hypertrophic osteoarthropathy or pachydermoperiostosis is a rare hereditary disorder characterized by digital clubbing, pachydermia and periostosis. Its precise incidence and prevalence is still unknown due to the lack of controlled data. It occurs without any underlying causes and usually has a chronic course. Life expectancy may be of normal standards, but many patients develop multiple functional and cosmetic complications. So, it is important to diagnose this disease at an early stage and to treat the symptomat for the quality of life. We report a case of primary hypertrophic osteoarthropathy in a 68-year-old male with clinical features such as digital clubbing and pachydermia, radiographic findings of acroosteolysis and periosteal new bone formation.

Keyword

Primary hypertrophic osteoarthropathy; Acroosteolysis

MeSH Terms

Acro-Osteolysis
Aged
Humans
Incidence
Life Expectancy
Male
Osteoarthropathy, Primary Hypertrophic
Quality of Life

Figure

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  Figure 1. Digital clubbing on both hands and the amputated 5 th mid phalanx of left hand are seen.

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  Figure 2. (A) Plain radiograph of both hands demonstrate acroosteolysis. Extensive bone lysis of distal phalanges with bases for the preservation are seen. (B) Plain radiograph of lower extremities shows irregular linear cortical thickening of both proximal tibia (white arrows) and periosteal new bone formation.

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  Figure 3. Tc-99m HDP scan finding shows diffuse and symmetrical increased uptakes of radionuclide in a linear pattern along the periosteal surfaces of both proximal tibia.

Reference

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