Keratosis palmoplantaris papulosa

A rare autosomal dominant hereditary form of palmoplantar keratoderma characterized by multiple hyperkeratotic centrally indented papules that develop in early adolescence or later and are irregularly distributed on the palms and soles. In mechanically irritated areas, confluent plaques can be found. Interfamilial and intrafamilial severity shows broad variation. It may be associated with the development of early- and late-onset malignancies, including SQUAMOUS CELL CARCINOMA. Mutations in the AAGAB gene have been identified. OMIM: 148600

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