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Folliculotropic mycosis fungoides (stage IIA) progressing to Sézary syndrome: a case report
Author(s) -
Agar N.,
Whittaker S.J.
Publication year - 2008
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.2008.08802.x
Subject(s) - mycosis fungoides , stage (stratigraphy) , medicine , clone (java method) , dermatology , peripheral t cell lymphoma , trunk , lymphoma , pathology , cutaneous t cell lymphoma , atypical lymphocyte , immunology , biology , t cell , gene , immune system , paleontology , ecology , biochemistry
Summary Folliculotropic mycosis fungoides is associated with a worse prognosis than classical mycosis fungoides (MF), but whether this is due to resistance to skin‐directed therapy or to biological differences is unclear. We discuss a case of a patient with folliculotropic MF (stage IIA) who progressed to develop Sézary syndrome (SS), stage IVB, over 6 years. A 40‐year‐old man presented with pruritic plaques affecting his head and trunk, characterized by follicular plugging. The histology was consistent with folliculotropic MF and T‐cell gene analysis studies revealed a T‐cell clone in the skin only. His condition gradually deteriorated and 5 years after presentation, T‐cell gene analysis studies revealed the presence of a clone in the blood identical with that seen in the skin. His condition progressed with the development of erythrodermic disease and a leukaemic blood picture and he subsequently died of systemic nodal and visceral involvement. We present the first report detailing the stepwise progression of a patient with stage IIA folliculotropic MF to SS. This case demonstrates that MF and SS represent a clinical spectrum of the same disease.