By Gary M. White, MD
Loricrin keratoderma (LK) is a rare autosomal dominant genodermatosis characterized by palmoplantar keratoderma and ichthyosis.
- LK is caused by a mutation in loricrin, an important protein in epidermal maturation.
- LK may present at birth as a collodion baby.
- During childhood, honey-comb palmoplantar keratoderma (PPK) and a diffuse mild ichthyosis may develop.
- The PPK has honeycomb-like features, similar to Vohwinkel (formerly Camisa variant of autosomal dominant Vohwinkel syndrome)
- In contrast to Vohwinkel syndrome, there is generalized ichthyosis, but there is no deafness.
A 14‐year‐old boy presented with generalized dryness and scaling affecting his trunk and all four limbs, previously thought to be ichthyosis vulgaris, which was reported from early childhood examination revealed widespread, prominent ichthyosis and mild diffuse transgredient hyperkeratosis of palms and soles. His mother and eight other family members were similarly affected. Br J Dermatol. 2015 Nov;173(5):1291-4
Palmar honeycomb pattern of keratoderma. JAAD October 2010 Volume 63, Issue 4, Pages 607–641
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