Howell-Evans Syndrome (cancro esofágico)

  • AD
  • Defect in TOC locus
  • Focal painful PPK over pressure points
  • Increased risk esophageal cancer
    • Type A: late onset PPK, higher risk of esophageal carcinoma
    • Type B: early onset PPK, benign course
  • Tx: topicals, oral retinoids, refer to GI