Open AccessScleromyxedematous Changes in a Patient With Long-Standing Mycosis Fungoides Who Progressed to Sézary Syndrome
Author(s) -
Allen P. Miraflor,
Melissa Pulitzer,
Patricia L. Myskowski,
Shamir Geller
Publication year - 2019
Publication title -
the american journal of dermatopathology/american journal of dermatopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.454
H-Index - 73
eISSN - 1533-0311
pISSN - 0193-1091
DOI - 10.1097/dad.0000000000001464
Subject(s) - mycosis fungoides , medicine , dermatology , mucinosis , pathology , peripheral t cell lymphoma , mucin , immunohistochemistry , radiation therapy , lymphoma , immune system , immunology , t cell
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.