FaCD Online Syndrome Fact Sheet

Last updated: 01 Sep 2008

Name: Punctate Palmoplantar Keratoderma

Synonym: Buschke-Fischer-Brauer disease, Keratosis Palmoplantaris Papulosa

Mode of Inheritance: AD

Tumor features (possible)

colon cancer

Non-tumor features

palmoplantar hyperkeratosis

Comment

Acquired punctate keratoses has been found in association with internal malignancies[1;2]. In a few families with hereditary punctate palmoplantar keratoderma, a few cases of early onset (43, 46, 53 years) colon cancer have been observed[3;4]. Other tumors were reported as well: pancreatic cancer, Hodgkin’s disease, renal and breast cancer, but these developed at an age similar too that seen in sporadic tumors.[5,6,7]. No evidence for increased cancer risk was reported by Emmert et al who reviewed 47 patients from 14 punctate palmoplantar keratoderma families[8].

References

[1] Dobson RL, Young MR, Pinto JS. Palmar keratoses and cancer. Arch Dermatol 1965; 92:553-556.
[2] Fegueux S, Bilet S, Crickx B, Perron J, Grossin M, Belaïch S. Hyperkératose palmo-plantaire filiforme et cancer recto-sïgmoidien. Ann Dermatol Venereol 1988; 115:1145-1146.
[3] Bennion SD, Patterson JW. Keratosis punctata palmaris et plantaris and adenocarcinoma of the colon: a possible familial association of punctate keratoderma and gastrointestinal malignancy. J Am Acad Dermatol 1984; 10:587-591.
[4] Ena P, Cottoni F, Cerimele D, Saccabusi S, Retanda G. Associazione della cheratodermia palmo-plantare punctata con altre condizioni morbose (canizie precoce, carcinoma del colon): studio su tre famiglie. Giorn Ital dermat vener 1986; 121:45-54.
[5] Stevens HP, Kelsell DP, Leigh IM, Ostlere LS, MacDermot KD, Rustin MH. Punctate palmoplantar keratoderma and malignancy in a four-generation family. The British journal of dermatology 1996; 134(4):720-6.
[6] Hillion B, le Bozec P, Moulonguet-Michau I, Blanchet-Bardon C, Petit A, Stephan J, Civatte J. Hyperkératose palmo-plant filiforme et cancer du sein. Ann Dermatol Venereol 1990; 117:834-836.
[7] Bianchi L, Orlandi A, Iraci S, Spagnoli LG, Nini G. Punctate porokeratotic keratoderma--its occurrence with internal neoplasia. Clinical and experimental dermatology 1994; 19(2):139-41.
[8] Emmert S, Küster W, Hennies HC, Zutt M, Haenssle H, Kretschmer L, Neumann C. 47 patients in 14 families with the rare genodermatosis keratosis punctata palmoplantaris Buschke-Fischer-Brauer. European journal of dermatology : EJD 2003 Jan-Feb; 13(1):16-20.