PACHYDERMOPERIOSTOSIS

Pachydermoperiostosis is a syndrome characterized by hypertrophy of the skin and soft tissue as well as bony changes. In the primary form, onset is soon after puberty. Secondary pachydermoperiostosis is usually a paraneoplastic process secondary to a pulmonary neoplasm. It is most common therefore in adult men. In the secondary form, the clubbing and bone changes are most prominent and the skin folding may be slight or non-existent.

Clinical

In the primary form, the skin of the forehead and scalp and to some extent the rest of the face becomes thickened and enlarged to the point of undulating with deep furrows and gyrate patterns, reminiscent of the surface of the brain. The folding of the skin of the scalp is called cutis verticis gyrata. (Pachydermoperiostosis is one cause of cutis verticis gyrata, but CVG more frequently occurs in isolation.) Sebaceous activity of the face is increased and there may be hyperhidrosis of the palms and soles.

Clubbing of the fingers and toes is also a characteristic finding. The bones of the arms and legs may be thickened. Radiographs may show periosteal thickening as well as some thickening of the cortex.

Treatment

In the primary form, plastic surgery may be consulted.

References

  1. Medicine 1964;43;459

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