Familial pachydermoperiostosis in association with protein-losing enteropathy

Clin Exp Dermatol. 2006 Jul;31(4):531-4. doi: 10.1111/j.1365-2230.2006.02138.x.

Abstract

In this report we describe a rare association of pachydermoperiostosis with protein-losing enteropathy (PLE) in a family of three brothers. The first brother had the complete form of pachydermoperiostosis along with PLE. The second brother had the 'forme fruste' of pachydermoperiostosis, with minimal skin changes, bony abnormalities and PLE, which was due to intestinal lymphangiectasia. The third brother had an incomplete form of pachydermoperiostosis without evidence of PLE. To our knowledge, the association of pachydermoperiostosis with PLE due to intestinal lymphangiectasia has not been reported previously.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Humans
  • Lymphangiectasis, Intestinal / complications*
  • Male
  • Osteoarthropathy, Primary Hypertrophic / etiology*
  • Osteoarthropathy, Primary Hypertrophic / pathology
  • Protein-Losing Enteropathies / etiology*
  • Siblings