Odonto-onycho-dermal dysplasia (Schöpf– Schulz–Passarge Syndrome)

  • Wingless integrated family 10A gene mutation
  • Autosomal recessive inheritance
  • Impaired ectodermal “appendage” development
  • Diffuse palmoplantar keratoderma, facial telangiectasia / reticulate erythema, dental defects, nail dystrophy, hypotrichosis
  • Schöpf– Schulz–Passarge syndrome 
    • Eyelid hidrocystomas, adnexal tumours

Pathology: 

  • Eccrine syringofibroadenomas
  • Unknown