Keratosis palmplantaris papulosa Buschke-Fischer-Brauer

Buschke and Fischer 1910

Brewer 1913

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

A autosomal – dominant inheritance is assumed, although the mutated gene is not known.

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

Punctate hyperkeratosis palmoplantar.

Nail dystrophies.

Increased risk of lymphoma and solid tumours.

Anamnesis

Clinic

Tumour – Search

Keratolytic treatment.

Regular re-lubrication.

1. Charfeddine C, Ktaifi C, Laroussi N, Hammami H, Jmel H, Landoulsi Z, Badri T, Benmously R, Bchetnia M, Boubaker MS, Fenniche S, Abdelhak S, Mokni M. Clinical and molecular investigation of Buschke-Fischer-Brauer in consanguineous Tunisian families. J Eur Acad Dermatol Venereol. 2016 Dec;30(12):2122-2130. 
2. Giehl KA, Herzinger T, Wolff H, Sárdy M, von Braunmühl T, Dekeuleneer V, Sznajer Y, Tennstedt D, Boes P, Rapprich S, Wagner N, Betz RC, Braun-Falco M, Strom T, Ruzicka T, Eckstein GN. Eight Novel Mutations Confirm the Role of AAGAB in Punctate Palmoplantar Keratoderma Type 1 (Buschke-Fischer-Brauer) and Show Broad Phenotypic Variability. Acta Derm Venereol. 2016 May;96(4):468-72. 
3. Podder I, Das A, Bhattacharya S, Shome K, Chowdhury SN. Buschke-Fischer-Brauer Keratoderma: Linear Variety Associated with Hodgkin's Lymphoma. Indian J Dermatol. 2015 May-Jun;60(3):322.