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

Autor: Patricia L. Myskowski, Shamir Geller, Melissa Pulitzer, Allen P. Miraflor
Rok vydání: 2019
Předmět:
Zdroj: Am J Dermatopathol
ISSN: 0193-1091
DOI: 10.1097/dad.0000000000001464
Popis: 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 afterwards. Total skin electron beam (TSEB) radiation therapy resulted in an excellent response, controlling both the MF/SS and the scleromyxedematous lesions; however, the patient expired 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 require further investigation.
Databáze: OpenAIRE