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Scleromyxedematous Changes in a Patient With Long-Standing Mycosis Fungoides Who Progressed to Sézary Syndrome

Miraflor, Allen P. MD*; Pulitzer, Melissa P. MD*; Myskowski, Patricia L. MD; Geller, Shamir MD†,‡

The American Journal of Dermatopathology: November 2019 - Volume 41 - Issue 11 - p e139–e143
doi: 10.1097/DAD.0000000000001464
Extraordinary Case Report
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Abstract: Mycosis fungoides (MF) variants with different clinicopathologic and immunohistochemical features have been well-delineated. We report a case of scleromyxedematous changes arising in a patient with long-standing MF who progressed to Sézary syndrome (SS) shortly afterward. Total-skin electron-beam radiation therapy resulted in an excellent response, controlling both the MF/SS and the scleromyxedematous lesions; however, the patient died few months later. Although mucin deposition has been described in association with MF/SS (mainly follicular mucinosis in folliculotropic MF), there are limited reports in the literature on dermal mucinosis and scleromyxedematous changes in MF/SS. The mechanism of this association and its prognostic implications requires further investigation.

*Department of Pathology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medicine, New York, NY;

Dermatology Service, Division of Subspecialty Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medicine, New York, NY; and

Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.

Correspondence: Shamir Geller, MD, Dermatology Service, Division of Subspecialty Medicine, Memorial Sloan Kettering Cancer Center, 16 East 60th Street, New York, NY 10022 (e-mail: shamirgeller@gmail.com).

The present research was funded in part through the National Institutes of Health, National Cancer Institute Cancer Center Support Grant P30 CA008748.

The authors declare no conflicts of interest.

This case was presented at The American Society of Dermatopathology annual meeting; October 2017; Baltimore, MD.

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