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 |
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Pages (from-to) | E139-E143 |
Journal | American Journal of Dermatopathology |
Volume | 41 |
Issue number | 11 |
DOIs | |
State | Published - 1 Nov 2019 |
Externally published | Yes |
Keywords
- cutaneous T-cell lymphoma
- dermal mucinosis
- fibrosis
- mycosis fungoides
- scleromyxedema