Sebaceous lesions and their associated syndromes: Part II

Sebaceous lesions are associated with two syndromes with widespread multisystem disorders and tumors. Linear sebaceous nevus syndrome has been traditionally known as the triad of sebaceous nevus of Jadassohn, seizures, and mental retardation. This syndrome encompasses a much broader spectrum of multisystem disorders, which is explored below. Muir–Torre syndrome is described as the presence of sebaceous tumors or keratoacanthomas with an underlying visceral malignancy. It is caused by mutations in DNA mismatch repair genes. We discuss its relationship with Lynch syndrome and suggest a comprehensive algorithm on how to screen patients with sebaceous neoplasms for Muire–Torre syndrome. We also provide suggested intensive cancer screening guidelines based on recommendations for patients with Lynch syndrome that may also be of value for patients with Muir–Torre syndrome.Learning objectivesAfter completing this learning activity, participants should be able to discuss the characteristics of Lynch and Muir–Torre syndromes, both of which are associated with sebaceous lesions, evaluate a patient who might have epidermal nevus syndrome, formulate an approach to identify patients at risk for Muir–Torre syndrome who have a newly diagnosed sebaceous neoplasm, and discuss screening recommendations for patients who are identified as having Muir–Torre syndrome.