| Acrochordons are not a component of the Birt-Hogg-Dubé syndrome: does this syndrome exist? Case reports and review of the literature. | |
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MedLine Citation:
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PMID: 10446780 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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C De la Torre; C Ocampo; I G Doval; A Losada; M J Cruces |
Publication Detail:
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Type: Case Reports; Journal Article; Review |
Journal Detail:
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Title: The American Journal of dermatopathology Volume: 21 ISSN: 0193-1091 ISO Abbreviation: Am J Dermatopathol Publication Date: 1999 Aug |
Date Detail:
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Created Date: 1999-09-17 Completed Date: 1999-09-17 Revised Date: 2005-11-16 |
Medline Journal Info:
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Nlm Unique ID: 7911005 Medline TA: Am J Dermatopathol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 369-74 Citation Subset: IM |
Affiliation:
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Service of Dermatology, Hospital Provincial, Pontevedra, Spain. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Female Fibroma / genetics, pathology* Hair Follicle / pathology Hamartoma / pathology* Humans Male Middle Aged Neoplasms, Multiple Primary / genetics, pathology* Skin Neoplasms / genetics, pathology* Syndrome |
| Comments/Corrections | |
Comment In:
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Am J Dermatopathol. 2000 Jun;22(3):293-4
[PMID:
10871079
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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