Muir-Torre syndrome-is it really a new syndrome?
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.
Grzybowski
Andrzej
A
The Poznań Society of Friends of Learning, Poland. jbhawan@bu.edu
Jabłońska
Stefania
S
D049672:History, 19th Century; D006801:Humans; D055653:Muir-Torre Syndrome / Q000266:history* / Q000473:pathology*