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Case Reports Medical Education Research Forum 2019
5-2019 A Man with Multiple Ulcerated Papules on His Face: A Muir-Torre Story Ethan Sagher Henry Ford Health System
David Oberlin Henry Ford Health System
Ellen Pritchett Henry Ford Health System
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Recommended Citation Sagher, Ethan; Oberlin, David; and Pritchett, Ellen, "A Man with Multiple Ulcerated Papules on His Face: A Muir-Torre Story" (2019). Case Reports. 14. https://scholarlycommons.henryford.com/merf2019caserpt/14
This Poster is brought to you for free and open access by the Medical Education Research Forum 2019 at Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Case Reports by an authorized administrator of Henry Ford Health System Scholarly Commons. For more information, please contact [email protected]. Ulcerated papules on the face: A Muir-Torre story Ethan Sagher MD, David Oberlin MD, and Ellen Pritchett, MD Department of Dermatology Henry Ford Health System, Detroit, MI
History Clinical Photos Discussion • A 61-year-old Caucasian man with a history of cardiac 3 4 • Muir-Torre syndrome is an autosomal dominant transplant in 2014 secondary to NICM presented to clinic for condition that is characterized by both cutaneous and full skin examination. visceral tumors. • His dermatologic history was significant for Muir-Torre syndrome (MTS) with MLH1 mutation on genetic testing. He • It is thought to be a subset of Lynch syndrome. had previously been treated surgically for multiple sebaceous • It is caused by mutations in genes that are responsible carcinomas and adenomas. for DNA mismatch repair, including MLH1, MSH2, • Family history was notable for colon cancer in his mother, MSH6, and PMS2. maternal grandfather, and maternal uncle, as well as uterine • Further testing may include microsatellite instability cancer in a cousin on his mother’s side. and/or germline mutation analysis. • Sebaceous neoplasms, commonly on the head, neck, Examination and trunk, are the most typical dermatologic 5 6 manifestation. Keratoacanthomas may also be seen. • Right and left forehead, and left cheek, with erythematous papules. Right forehead and left cheek lesions exhibited • Histology exhibits hypercellularity with poorly central ulceration. Partial crown of vessels appearance on differentiated sebocytes. Mitoses and buckshot scatter dermoscopy. in the epidermis may be seen. • Multiple scattered yellow umbilicated papules across the face. • Immunostaining is negative for the proteins corresponding to the mutated gene. Histology • Internal malignancies are most commonly colorectal and urogenital. 1 • Cutaneous findings often proceed visceral findings. • Surgical removal is first-line treatment for cutaneous Figures 3-6: Forehead, cheeks, and lateral neck with erythematous papules, some of which had central ulceration and central dells. malignancies. Radiation therapy may also be considered as an adjunctive therapy. Course and Therapy • Given his history of Muir-Torre syndrome, our differential included References
sebaceous carcinoma, sebaceoma, and sebaceous adenoma. • Jones B, Oh C, Mangold E, Egan C. Muir-Torre syndrome: diagnostic and • Biopsy results were consistent with sebaceous carcinoma for all three lesions. screening guidelines. Australas J Dermatol 2006; 47: 266-269. 2 • Ossama A, Mahalingam M. Cutaneous sebaceous neoplasms as markers of • The patient was referred to Mohs micrographic surgery, where the tumors Muir-Torre syndrome: a diagnostic algorithm. J Cutan Pathol 2009; 36: 613- Figure 1: Shave biopsy of a papule on were removed one layer at a time until all sections were clear. 619. the forehead. Hematoxylin and eosin • The patient continues to follow with dermatology closely, and frequent skin • Finan MC, Connolly SM. Sebaceous gland tumors and systemic disease: a staining revealed hypercellular islands of exams have since found more sebaceous neoplasms that have been treated clinicopathologic analysis. Medicine (Baltimore) 1984; 63: 232-42. poorly differentiated sebocytes with • Chang AY, Miller CJ, Elenitsas R, Newman JG, Sobanko JF. Management nuclear atypia and mitotic figures. surgically. considerations in extraocular sebaceous carcinoma. Dermatol Surg 2016; 42 • There was discussion about changing from tacrolimus to everolimus or suppl 1: S57-65. Figure 2: IHC (from the original biopsy) sirolimus to reduce his risk of skin cancer, but it was ultimately decided to • Husain A, Blumenschein G, Esmaeli B. Treatment and outcomes for showed negative staining for MLH1. metastatic sebaceous carcinoma of the eyelid. Int J Dermatol 2008; 47(3): keep his immunosuppressive regimen the same due to risk of transplant 276-279. rejection. • Levi Z, Hazazi R, Kedar-Barnes I, Hodak E, Gal E, Mor E, Niv Y, Winkler J. Switching from tacrolimus to sirolimus halts the appearance of new • He is monitored with regular colonoscopies and EGDs. A polyp was removed sebaceous neoplasms in Muir-Torre syndrome. Am J Transplant 2007; 7: during his most recent colonoscopy, found to be a tubular adenoma. 476-479