Identification of human papillomavirus DNA in cutaneous lesions of Cowden syndrome

Background: Cowden syndrome (CS) or multiple hamartoma syndrome is
a cancer-associated genodermatosis inherited in an autosomal
dominant pattern. One of the diagnostic criteria is facial papules
which are felt to be trichilemmomas, benign hair follicle tumors,
which some consider to be induced by human papillomavirus (HPV).
Objective: To search for HPV in skin tumors, especially
trichilemmomas, from patients with CS. Methods: Skin lesions from
patients with CS were classified histologically. Each tumor was
then analyzed for HPV DNA by polymerase chain reaction with
different primer sets; positive amplicons were typed by direct
sequencing. Results: Twenty-nine biopsies from 7 patients with CS
were investigated. Only 2 of 29 tumors clinically suspected of
being trichilemmomas were confirmed histologically. In addition, 3
sclerotic fibromas, also typical of CS, were found, as well as 1
sebaceous hyperplasia. The other 23 lesions showed histological
features of HPV-induced tumors in various stages of development.
HPV DNA was found in 19 of 29 cutaneous lesions. Tumors without any
histological signs of HPV induction were negative for HPV DNA. Two
tumors which were histologically classified as common warts
contained HPV types 27 and 28. All the 17 other HPV types belong to
the group of epidermodysplasia-verruciformis-associated types.
Conclusions: The majority of cutaneous lesions in CS contain HPV
DNA. They may have a variety of histological patterns.
Trichilemmomas are not clinically distinctive and can be difficult
to identify in CS patients. Copyright (C) 2003 S. Karger AG, Basel.