Premium
The clinicopathological features of familial cylindromas and trichoepitheliomas (Brooke‐Spiegler syndrome): a report of two families
Author(s) -
BURROWS N.P.,
RUSSELL JONES R.,
SMITH N.P.
Publication year - 1992
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/j.1365-2230.1992.tb00223.x
Subject(s) - penetrance , trichoepithelioma , pathological , dermatology , inheritance (genetic algorithm) , medicine , pathology , biology , genetics , phenotype , basal cell , gene , basal cell carcinoma
Summary We have studied four patients from two families and obtained the clinical data from four other affected family members who demonstrated the coexistence of multiple cylindromas and trichoepitheliomas. An autosomal dominant pattern of inheritance with variable penetrance was exhibited in both families, with a female to male ratio of 3:1. The clinical and pathological features of this rare association are discussed.