MeSH Browser

MeSH Supplementary: Keratosis palmoplantaris papulosa
Unique ID: C536161
RDF Unique Identifier: http://id.nlm.nih.gov/mesh/C536161
Entry Term(s): Buschke Fischer Brauer syndrome; Keratoderma, Palmoplantar, Punctate Type I; Keratoderma, palmoplantar punctate type 1; Keratodermia palmoplantaris papulosa, Buschke-Fischer-Brauer type; Type I punctate palmoplantar keratoderma
Registry Number: 0
Heading Mapped to: *Keratoderma, Palmoplantar
Frequency: 18
Note: 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
Date of Entry: 2010/08/25
Revision Date: 2015/09/26

Keratosis palmoplantaris papulosa MeSH Supplementary Concept Data 2024