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Loricrin Keratoderma

  • Loricrin (glycine-rich cornified envelope protein) gene mutation

  • Abnormal loricrin transport leads to altered cornification

  • Generalized desquamation or “collodion baby” at delivery with subsequent ichthyosis
  • Diffuse non-epidermolytic palmoplantar keratoderma with “honeycomb pattern”
    • pseudoainhum, knuckle pads, warty keratoses on extensors among children as in Vohwinkel syndrome without deafness

Pathology: 

  • Hyperkeratosis, parakeratosis, transitional cells, wide stratum granulosum, focal perinuclear vacuolization
    • Nuclear immunoreactivity 

Differential:

  • Ichthyosis hystrix Curth-Macklin

  • Isotretinoin