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A Case of Bullous Sézary Syndrome
Author(s) -
Ono Atsuko,
Isomura Iwao,
Isogai Zenzo,
Shintani Youichi,
Suzuki Akiko,
Morita Akimichi
Publication year - 2004
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2004.tb00649.x
Subject(s) - erythroderma , mycosis fungoides , medicine , pathology , infiltration (hvac) , atypical lymphocyte , lymphoma , dermatology , peripheral t cell lymphoma , cutaneous lymphoma , t cell , immune system , immunology , physics , thermodynamics
Sézary syndrome is an aggressive variant of cutaneous T cell lymphoma with poor prognosis and clinically characterized by erythroderma and Sézary cells in the blood. Here we report a case of bullous Sézary syndrome. A seventy‐year‐old male presented with erythroderma and inguinal lymph node swelling. Histopathological examination showed dermal and epidermal infiltration of atypical lymphocytes and Sézary cells could be detected in peripheral blood samples. He was therefore diagnosed as Sézary syndrome. Four months after the onset, he developed bullae on axillary and inguinal areas, featuring subepidermal blistering with basal cell degeneration and dense infiltration of atypical lymphocytes. Autoimmune bullous diseases were excluded by negative immunofluorescence. Bullous forms of Sézary syndrome are extremely rare although several cases of a bullous variant of mycosis fungoides have been reported.