title: Acrochordons are not a component of the Birt-Hogg-Dubé syndrome: does this syndrome exist? Case reports and review of the literature.
abstract: Multiple fibrofolliculomas, trichodiscomas, and acrochordons compose the triad of cutaneous lesions characterizing the Birt-Hogg-Dubé syndrome, inherited in an autosomal dominant fashion. We report a case of a family who had the triad of tumors of the Birt-Hogg-Dubé syndrome. Two members were observed clinically and histologically. Biopsies of the facial papules disclosed features of the fibrofolliculoma/trichodiscoma spectrum. Lesions that were clinically acrochordon-like proved to correspond to the same histopathologic spectrum. The characterization of the nature of the acrochordon-like lesions in our patients and the review of the literature allow us to question if acrochordons (skin tags) should be maintained as a component of this association. In light of our conclusion that fibrofolliculoma, trichodiscoma, and the acrochordon-like lesions are histologic variations of a single lesion, we further question whether the term "syndrome" is valid.
authors.affiliation: Service of Dermatology, Hospital Provincial, Pontevedra, Spain.
authors.forename: C
authors.initials: C
authors.lastname: De la Torre
authors.forename: C
authors.initials: C
authors.lastname: Ocampo
authors.forename: I G
authors.initials: IG
authors.lastname: Doval
authors.forename: A
authors.initials: A
authors.lastname: Losada
authors.forename: M J
authors.initials: MJ
authors.lastname: Cruces
mesh_terms: D005260:Female; D005350:Fibroma / Q000235:genetics / Q000473:pathology*; D018859:Hair Follicle / Q000473:pathology; D006222:Hamartoma / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000235:genetics / Q000473:pathology*; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology*; D013577:Syndrome