Pachydermoperiostosis

Case contributed by Dr Yasser Asiri

Presentation

Longstanding history of progressive enlargement of the extremities and coarse facial features.

Patient Data

Age: 50 years
Gender: Male
X-ray

Skeletal survey showed diffuse bilateral periosteal new bone formation and symmetrical cortical thickening notable along the lower and upper extremity long bones, clubbing of the fingers can be appreciated as well. The overall findings are consistent with pachydermoperiostosis.

Case Discussion

Pachydermoperiostosis is also known as primary hypertrophic osteoarthropathy and is characterized by symmetrical periosteal bone formation in the tibia, fibula, radius and ulna. Clinically it presents with thickening of the skin and coarsening of facial features. 

This patient was admitted for further investigation and work-up to rule out a secondary form of hypertrophic osteoarthropathy and all tests were unremarkable. The patient's brother has been diagnosed with the same disease suggesting a familial form and further genetic testing has been requested.

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Case information

rID: 65048
Published: 20th Dec 2018
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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