Mycosis fungoides/Sézary syndrome: report of an unusual case.
BACKGROUND
Sézary syndrome (SS) is an uncommon form of cutaneous T cell lymphoma (CTCL) with a classical triad of lymphadenopathy, characteristic circulating lymphoma cells (Sézary cells) and erythrodermatous skin involvement with classical mycosis fungoides (MF)-like histological picture.


METHODS
A 32-year-old woman presented with this classical triad; however, her skin involvement, histologically, was in the form of folliculotropic MF, rather than the usual classical form of MF.


CONCLUSIONS
In the vast majority of cases, the cutaneous involvement in SS resembles conventional MF, histologically. One case of CD30-positive CTCL with pilotropic MF has been reported. However, English literature does not describe any case of SS with folliculotropic MF with typical immunophenotype of SS thus far. We presume that this case represents the first report of SS with folliculotropic MF histologically, displaying the typical CD30-negative immunophenotype.
Mehta
Anurag
A
Department of Pathology, Armed Forces Medical College, Pune, Maharastra, India.
Dhungel
Bal Makunda
BM
Khan
Mohd Fawad Farooq
MF
D000328:Adult; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D017730:Ki-1 Antigen / Q000276:immunology; D009182:Mycosis Fungoides / Q000276:immunology / Q000473:pathology*; D012751:Sezary Syndrome / Q000276:immunology / Q000473:pathology*; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology*
D017730:Ki-1 Antigen