A case of bullous Sézary syndrome.
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.
Ono
Atsuko
A
Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences, 1-Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan.
Isomura
Iwao
I
Isogai
Zenzo
Z
Shintani
Youichi
Y
Suzuki
Akiko
A
Morita
Akimichi
A
D000368:Aged; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D012751:Sezary Syndrome / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D012872:Skin Diseases, Vesiculobullous / Q000097:blood / Q000175:diagnosis / Q000473:pathology; D012878:Skin Neoplasms / Q000097:blood / Q000175:diagnosis* / Q000473:pathology