Medication Summary

The goals of pharmacotherapy for Muir-Torre syndrome (MTS) are to prevent development of neoplasms, reduce morbidity, and prevent complications.

Class Summary

These agents regulate cell growth and proliferation. They may prevent some of the neoplasms in MTS.

Isotretinoin (Amnesteem, Claravis, Myorisan, Absorica)

View full drug information

Isotretinoin is an oral agent that treats serious dermatologic conditions. It is a synthetic 13-cis isomer of naturally occurring tretinoin (trans- retinoic acid). Both agents are structurally related to vitamin A.

A US Food and Drug Administration–mandated registry is now in place for all individuals prescribing, dispensing, or taking isotretinoin. For more information on this registry, see iPLEDGE. This registry aims to further decrease the risk of pregnancy and other unwanted and potentially dangerous adverse effects during a course of isotretinoin therapy.

Questions

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Media Gallery

Sebaceous carcinoma of the upper eyelid. Courtesy of Mark S. Brown, MD, University of South Alabama Medical Center.
Sebaceous carcinoma as viewed from the conjunctival side. Courtesy of Mark S. Brown, MD, University of South Alabama Medical Center.
Gross image of an ear resection due to a deeply invasive sebaceous carcinoma.
Histologic section of sebaceous adenoma showing a predominance of sebaceous cells with prominent cytoplasmic vacuoles.
Histologic section of sebaceous epithelioma showing a predominance of basaloid cells.
Well-differentiated sebocytes in small nests, deeply infiltrating the subcutaneous tissue, thus consistent with sebaceous carcinoma.
Low-power view of a keratoacanthomalike sebaceous adenoma. Well-circumscribed, symmetrical lesion with overlying papillomatosis and prominent hyperkeratosis and showing focal sebaceous differentiation (arrows).
Sebaceous carcinoma typically infiltrates the overlying epithelium in a manner similar to Paget disease. Note in this case how the atypical cells have mostly replaced the normal follicular cells and involve the overlying epidermis.
Normal pattern of expression of MSH-2 (nuclear positivity) in a sebaceous carcinoma from a patient with Muir-Torre syndrome (see also MSH-6).
Significant loss of MSH-6 expression in this sebaceous carcinoma in a patient with Muir-Torre syndrome.

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Author

Victor G Prieto, MD, PhD Ferenc and Phyllis Gyorkey Chair for Research and Education in Pathology, Professor, Departments of Pathology and Dermatology, University of Texas MD Anderson Cancer Center

Victor G Prieto, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Medical Association, American Society for Clinical Pathology, American Society of Dermatopathology, College of American Pathologists, European Society of Pathology, International Society of Dermatopathology, Society for Investigative Dermatology, United States and Canadian Academy of Pathology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Myriad (consultant regarding MyPath test in melanocytic lesions).

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Joshua A Zeichner, MD Assistant Professor, Director of Cosmetic and Clinical Research, Mount Sinai School of Medicine; Chief of Dermatology, Institute for Family Health at North General

Joshua A Zeichner, MD is a member of the following medical societies: American Academy of Dermatology, National Psoriasis Foundation

Disclosure: Received consulting fee from Valeant for consulting; Received grant/research funds from Medicis for other; Received consulting fee from Galderma for consulting; Received consulting fee from Promius for consulting; Received consulting fee from Pharmaderm for consulting; Received consulting fee from Onset for consulting.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Marcelo G. Horenstein, MD, to the development and writing of this article.