Sézary syndrome presenting with 'leonine facies'

The Australasian Journal of Dermatology
Shano NassemNeeraj Kumari

Abstract

A 71-year-old man presented with erythroderma and multiple nodular skin lesions over the face, scalp, upper limbs and trunk. The facial skin was thickened, producing the rare 'leonine facies' appearance. Investigations revealed the presence of atypical lymphoid cells in the peripheral blood, bone marrow and skin. The atypical lymphoid cells in the peripheral blood and bone marrow were positive for helper T-cell antigens (CD4, CD2, CD5 and CD7) on immunophenotyping by flow cytometry. The histopathology of skin showed dermal infiltration by atypical small lymphocytes with epidermotropism. These cells were positive for helper T-lymphocyte antigens on immunohistochemistry. A diagnosis of Sézary syndrome was made based on clinical, peripheral blood and immunophenotypical findings.

References

Jan 5, 2002·Journal of the American Academy of Dermatology·Eric C VonderheidUNKNOWN ISCL
Jan 9, 2004·Hematology/oncology Clinics of North America·Marie-France DemierreRein Willemze
Feb 5, 2005·Blood·Rein WillemzeChris J L M Meijer
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Oct 24, 2007·Critical Reviews in Oncology/hematology·Pier Luigi ZinzaniLorenzo Cerroni
Mar 18, 2008·Lancet·Sam T HwangWyndham H Wilson

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Citations

Nov 22, 2012·Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG·Nina BookenClaus-Detlev Klemke
Jan 19, 2020·American Journal of Clinical Dermatology·Lisa MorrisMadeleine Duvic
Aug 15, 2015·Journal of Cutaneous Pathology·Chee Won OhCarlos Torres-Cabala

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