Focal Palmoplantar Keraoderma without Associated Features
Punctate Palmoplantar Keratoderma with Associated Features Syndromic
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Howel–Evans Syndrome

  • Rhomboid 5 homolog 2 gene mutation
  • Autosomal dominant inheritance
  • Altered intramembrane protease function leading to increased epidermal growth factor receptor activity
    • Epithelial hyperproliferation
    • Aberrant wound healing
  • Focal palmoplantar keratoderma at pressure sites (plantar more than palmar), keratosis pilaris, rough / xerotic skin, oral leukokeratosis around 5-15 years of age
  • Increased risk for esophageal carcinoma by 50 years of age
  • Unknown