Premium
Punctate palmoplantar keratoderma and malignancy in a four‐generation family
Author(s) -
STEVENS H.P.,
KELSKLL D.P.,
LEIGH I.M.,
OSTLERE L.S.,
MACDERMOT K.D.,
RUSTIN M.H.A.
Publication year - 1996
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.1996.84797.x
Subject(s) - malignancy , penetrance , palmoplantar keratoderma , keratoderma , medicine , pathology , dermatology , hyperkeratosis , biology , genetics , phenotype , gene
Summary We report a large kindred in which a punctate palmoplantar keratoderma (PPK) is associated with malignancy, including Hodgkin's disease, renal, breast, pancreatic and colonic adenocarcinomas. The family was traced through four generations, and over 520 individuals were identified, of whom 49 had punctate PPK. The punctate PPK appeared to be inherited as an autosomal dominant trait with variable penetrance. Ten of the 43 adults (23%) with punctate PPK developed malignancies, and five of these developed before the age of 50. Of the 271 unaffected individuals, six (2%) have developed malignancies, one prior to the age of 50. The association of keratoderma and malignancy is discussed.