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Sézary syndrome presenting with ‘leonine facies’
Author(s) -
Nassem Shano,
Kashyap Rajesh,
Awasthi Namrata P,
Krishnani Narendra,
Kumari Neeraj
Publication year - 2009
Publication title -
australasian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.67
H-Index - 53
eISSN - 1440-0960
pISSN - 0004-8380
DOI - 10.1111/j.1440-0960.2009.00560.x
Subject(s) - medicine , pathology , immunophenotyping , bone marrow , cd5 , skin biopsy , antigen , biopsy , immunology , lymphoma
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.