Loricrin keratoderma
Loricrin keratoderma
Definition
A diffuse palmoplantar keratoderma, characterized by honeycomb palmoplantar hyperkeratosis associated with pseudoainhum of the fifth digit of the hand, ichthyosis and deafness. Keratoderma hereditarium mutilans with ichthyosis follows an autosomal dominant mode of transmission.
Also known as Camisa disease, keratoderma hereditarium mutilans with ichthyosis, keratoderma-ichthyosiform dermatosis-elevated beta-glucuronidase syndrome, loricrin keratoderma, Vohwinkel syndrome with ichthyosis — per MONDO