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

Allen P. Miraflor; Melissa P. Pulitzer; Patricia L. Myskowski; Shamir Geller

doi:10.1097/DAD.0000000000001464

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

Allen P. Miraflor, Melissa P. Pulitzer, Patricia L. Myskowski, Shamir Geller^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

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.

Original language	English
Pages (from-to)	E139-E143
Journal	American Journal of Dermatopathology
Volume	41
Issue number	11
DOIs	https://doi.org/10.1097/DAD.0000000000001464
State	Published - 1 Nov 2019
Externally published	Yes

Funding

Funders	Funder number
National Cancer Institute	P30CA008748

Keywords

cutaneous T-cell lymphoma
dermal mucinosis
fibrosis
mycosis fungoides
scleromyxedema

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1097/DAD.0000000000001464

Cite this

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title = "Scleromyxedematous Changes in a Patient with Long-Standing Mycosis Fungoides Who Progressed to S{\'e}zary Syndrome",

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 S{\'e}zary 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.",

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AU - Pulitzer, Melissa P.

AU - Myskowski, Patricia L.

AU - Geller, Shamir

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N2 - 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.

AB - 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.

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KW - fibrosis

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KW - scleromyxedema

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

Abstract

Funding

Keywords

UN SDGs

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