Focal Palmoplantar Keraoderma without Associated Features
Punctate Palmoplantar Keratoderma with Associated Features Syndromic
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Papillon–Lefèvre and Haim–Munk Syndromes

  • Cathepsin gene mutation
  • Autosomal recessive inheritance
  • Abnormal cutaneous / immune cell serine protease activation
  • Diffuse, transgredient palmoplantar keratoderma, erythema, pseudoainhum, hyperkeratotic psoriasiform plaques, childhood periodontitis with tooth loss
  • Increased risk of pyogenic infection and hepatic abscess, acral melanoma, squamous cell carcinoma
  • Haim–Munk syndrome
    • Onychogryphosis, arachnodactyly, acro-osteolysis
  • Oral retinoids decrease hyperkeratosis and risk of infection