原发性干燥综合征合并蕈样肉芽肿1例

A Case of Primary Sjogren's Syndrome with Mycosis Fungoides

患者男,54岁,汉族,10余年前无明显诱因腹部出现红斑,伴明显瘙痒。皮肤科情况:躯干、四肢大小不等暗红色、褐色斑片,色素分布不均匀,边界不清,表面干燥、肥厚,轻度浸润,唇部干裂。白细胞3.02×10^(9)/L↓,血沉26 mm/h↑,补体C30.69 g/L↓;自身抗体:抗SSA、RO-52抗体强阳性,ANA 1:320颗粒型。皮损组织病理示:真皮浅层可见大量淋巴细胞呈带状浸润,可见明显的淋巴细胞亲表皮现象,局部表皮内可见Pautrier微脓肿,部分细胞核大深染。诊断:原发性干燥综合征合并蕈样肉芽肿。

A 54-year-old male,Han nationality,presented with abdominal erythema without obvious triggers more than 10 years,accompanied by obvious pruritus.Physical examination showed that dark red and brown patches varied in size in trunk and limbs,uneven distribution of pigmentation,unclear boundary,dry hypertrophic surface,mild infiltration,and dry and cracked lip.WBC 3.02×10^(9)/L↓,ESR 26 mm/h↑,complement C30.69 g/L↓.Autoantibodies:anti SSA,Ro-52 antibodies were strongly positive,ANA 1:320 granular type.Histopathological examination showed that a large number of lymphocytes were infiltrated in the superficial dermis,and obvious phenomenon of lymphocyte epidermotrophism was observed.Pautrier micro abscess was found in the local epidermis,and some nuclei were large and hyperchromatic.Diagnosis:primary Sjogren's syndrome with mycosis fungoides.