Pachydermoperiostosis

Pachydermoperiostosis (PDP) refers to rare osteo-arthro-dermopathic syndrome with familial and idiopathic forms. It is also known as primary hypertrophic osteoarthropathy because it is not due to a secondary cause such as lung disease.

Also known as Touraine-Solente-Golé syndrome (named after the three French physicians who described it in 1935).

PDP most commonly affects black people and adolescent boys.

PDP is characterised by marked thickening of the skin of the extremities, face, and scalp. 

PDP is an autosomal dominant inherited disorder that is self-limiting and progresses for several years by stabilising. 

Plain radiograph/CT

Skeletal findings include symmetric, shaggy subperiosteal bone formation in the long bones, especially of the forearm and leg bones, but also of the metacarpals, metatarsals, and phalanges.

Involvement of the epiphyseal region distinguishes it from the secondary form, in which the epiphyses are usually spared 2.

There can be a widening of the ends of bones, especially in the wrist and knee joints which are due to increased bone formation. The generalised and symmetric periosteal reaction in pachydermoperiostosis tends to blend with the cortex and primarily involves the distal ends of the radius, ulna, tibia, and fibula.

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Article Information

rID: 18570
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Primary hypertrophic osteoarthropathy
  • Pachydermoperiostosis (PDP)

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