Department of Hematology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Australas J Dermatol. 2009 Nov;50(4):285-8. doi: 10.1111/j.1440-0960.2009.00560.x.
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.
一位 71 岁男性以红皮病和面部、头皮、上肢和躯干多发性结节性皮损为表现就诊。面部皮肤增厚，呈现罕见的“狮子脸”外观。检查发现外周血、骨髓和皮肤中存在异型淋巴细胞。外周血和骨髓中的异型淋巴细胞通过流式细胞术免疫表型分析呈辅助 T 细胞抗原阳性（CD4、CD2、CD5 和 CD7）。皮肤组织病理学显示真皮浸润有表皮倾向的异型小淋巴细胞。这些细胞在免疫组化上辅助 T 淋巴细胞抗原阳性。根据临床、外周血和免疫表型发现，诊断为蕈样肉芽肿。
