What is in a name-Perifollicular fibroma or fibrofolliculoma?
Publisher DOI
PubMed ID
37649357
Abstract
So far, confusion exists regarding the question of whether hereditary perifollicular fibromas and fibrofolliculomas can be distinguished from each other. Here, histopathological arguments are presented to clarify this terminological problem. In 1977, Birt et al. described a large kindred affected with hereditary multiple "fibrofolliculomas," which they thought were "a hitherto unrecognized pilar hamartoma," but they never claimed the fibrofolliculomas were part of a syndrome. A careful microscopic comparison shows, however, that the tumors are clinically and histopathologically identical to perifollicular fibromas, as first described by Burnier and Rejšek in 1925. Their familial occurrence was discovered in 1971 by Civatte and Le Tréguilly. Before 1977, the term "perifollicular fibroma" was used for these skin tumors. By contrast, Hornstein and Knickenberg described in 1975 perifollicular fibromas as a cutaneous marker of a syndrome characterized by a predisposition to colon cancer and pneumothorax. Later, two French groups erroneously proposed the term "Birt-Hogg-Dubé syndrome" to describe the co-occurrence of fibrofolliculomas, trichodiscomas, and acrochordons, which was contrary to what Birt et al. had in mind. Hence, today, we should discriminate between the hereditary nonsyndromic perifollicular fibromas, as documented by Civatte and Le Tréguilly and later by Birt et al., and the syndromic perifollicular fibromas, as delineated by Hornstein and Knickenberg.
Date Issued
2024-02
Publication Type
Article
Subject(s)
Subjects
Birt-Hogg-Dubé syndrome Hornstein-Knickenberg syndrome fibrofolliculoma histopathology nonsyndromic perifollicular fibromas perifollicular fibroma syndromic features syndromic perifollicular fibromas terminology trichodiscoma
Language(s)
en
Author(s)
Happle, Rudolf |
Additional Credits
Journal
Journal of cutaneous pathology
Publisher
Wiley-Blackwell
ISSN
0303-6987
Access(Rights)
restricted