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Genetics

Abbas O, Mahalingam M.

American University of Beirut Medical Center, Beirut, Lebanon.

 J Cutan Pathol 2009;36:613–9.

Editor’s note: Muir–Torre syndrome (MTS) is a rare autosomal dominant genodermatosis that is associated with sebaceous tumors (adenomas, epitheliomas, and carcinomas), keratoacanthomas, and internal malignancies (predominantly gastrointestinal and genito-urinary). It is caused by mutations in one or more DNA mismatch repair (MMR) genes, such as MSH-2, MLH-1, and MSH-6. Sebaceous tumors are variably associated with MTS, with an association of 25–60% for sebaceous adenoma (J Am Acad Dermatol 1995;33:90–104, J Cutan Pathol 2003;30:178–84, J Invest Dermatol 2003;120:858–64, Mod Pathol 2008;21:159–64).

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