The clinicopathological features of familial cylindromas and trichoepitheliomas (Brooke-Spiegler syndrome): a report of two families.
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.
Burrows
N P
NP
St John's Dermatology Centre, St Thomas' Hospital, London.
Jones
R R
RR
Smith
N P
NP
D000328:Adult; D000368:Aged; D003528:Carcinoma, Adenoid Cystic / Q000235:genetics / Q000473:pathology*; D005190:Family; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000235:genetics / Q000473:pathology*; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics / Q000473:pathology*; D010375:Pedigree; D012535:Scalp; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology*