| Muir-Torre syndrome-is it really a new syndrome? | |
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MedLine Citation:
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PMID: 19955878 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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E.G. Muir and D. Torre independently described widespread cutaneous changes associated with internal malignancy, which are presently known as the Muir-Torre syndrome. This syndrome is defined as the coexistence of sebaceous adenomas, sebaceus carcinomas, keratoacanthomas, and pedunculated tumors, some with lobulated structure. The cutaneous involvement (sebaceous gland tumor) is associated with at least a single internal malignancy; mostly colonorectal or genitourinary malignancies. The syndrome is believed to be very rare, but some cases seem to have been unrecognized or misdiagnosed. It is inherited as an autosomal dominant trait with a variable degree of penetrance. Although Muir and Torre described this syndrome in 1967/1968, we found a report on a very similar case as described by C. Hilton Fagge from Guy Hospital in London, which was published 100 years earlier. In this case, there were very abundant small tumors, some pedunculated, and some deeper ones, with a finely lobulated structure, containing "a hair follicle or the external dermal coat of the follicle." The lobulated structures developed from the sebaceous glands, which were larger than normal, and surrounded by abundant fibrous tissue. For this reason, these changes were described under the misleading name of Molluscum Fibrosum. The clinical description of this case, however, is excellent and enables the recognition of the Muir-Torre syndrome. |
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Authors:
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Andrzej Grzybowski; Stefania Jab??o??ska |
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Publication Detail:
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Type: Historical Article; Journal Article; Portraits |
Journal Detail:
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Title: The American Journal of dermatopathology Volume: 31 ISSN: 1533-0311 ISO Abbreviation: Am J Dermatopathol Publication Date: 2009 Dec |
Date Detail:
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Created Date: 2009-12-03 Completed Date: 2010-02-02 Revised Date: - |
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: 799-802 Citation Subset: IM |
Affiliation:
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The Pozna?? Society of Friends of Learning, Poland. jbhawan@bu.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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History, 19th Century Humans Muir-Torre Syndrome / history*, pathology* |
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