Leukoencephalopathy-palmoplantar keratoderma syndrome is a rare, genetic epidermal disease characterized by early childhood-onset of punctate palmoplantar keratoderma in association with adult-onset leukoencephalopathy manifested by progressive tetrapyramidal syndrome and cognitive deterioration. The disease is leukoencephalopathy-palmoplantar keratoderma syndrome (Monarch Disease Ontology identifier MONDO_0016545).