Keratosis palmoplantaris papulosa Buschke-Fischer-Brauer

Last Updated: 2019-08-26

Author(s): -

Buschke und Fischer 1910

Brauer 1913

Keratoderma, palmoplantar, punctate type IA. OMIM 148600.

Palmoplantar keratosis, which can be associated with an increased risk of malignancy.

Autosomal - dominant inheritance, mutations in the AAGAB gene.  Localization 15q23. This gene encodes the alpha- and gamma-adaptin-binding protein p34. It plays a role in membrane transport.

Usually in/after puberty, various symptoms appear for the first time.

Punctual hyperkeratoses palmoplantar.

Nail dystrophies.

Increased risk of lymphoma and solid tumours.

Anamnesis 

Clinic 

Tumor - Search

Keratolytic treatment.

Regular refatting.

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