Hypertrophic Osteoarthropathy | Pachydermoperiostosis

Etiology
notion image
  • Primary: pachydermoperiostosis
    • Rare
    • Hereditary (dominant or recessive)
    • Early onset
  • Secondary
    • Pulmonary
      • Pulmonary AV malformations
      • Cystic fibrosis
      • Malignancy: bronchial carcinoma
        • Bariéty and Coury syndrome: clubing of fingers + gynecomastia
      • Infection (TB)
      • Bronchiectasis
      • Pulmonary fibrosis
      • Sarcoidosis
    • Digestive disorders
      • Esophageal and/or gastric cancers
      • Inflammatory enteropathies
    • Hepatobiliary disorders
 
Mechanism
  • Unknown. Possibly linked to abnormalities in local tissue oxygenation, possibly related to primary impairment of pulmonary circulation
  • Hypertrophy of skin (epidermis, collagen) and its appendages (sweat and sebaceous glands)
  • And bone extremities - proliferative periostitis with irregular periosteal ossification
 
Clinical presentation
  • Clubbing of digits | hippocratic fingers
  • Skin: pachyodermia, cutiis verticis gyrata, nail bulging
  • Osteoarticular: periostosis, acroosteolysis, arthriti