Huriez Syndrome

  • Infant onset
  • Gene map corresponds to 4q23 chromosome
  • Autosomal dominant inheritance

  • Unknown

  • Diffuse hand scleroatrophy leading to sclerodactyly, absent dermatoglyphs
  • Mild palmoplantar keratoderma (general “parchment-like” skin), well-demarcated hyperkeratosis above pressure sites, hypoplastic nails
  • Squamous cell carcinoma at sites of atrophy

Pathology: 

  • Orthohyperkeratosis, acanthosis, hypergranulosis
  • Near absence of Langerhans cells

Differential:

  • Kindler syndrome
  • Dyskeratosis congenita
  • Palmoplantar keratoderma with sex reversal
  • Squamous cell carcinoma

  • Unknown