婴儿组织细胞型Sweet综合征1例

患儿男,6个半月。面颈部、躯干、四肢水肿性红斑、水疱伴痛25 d,加重伴间断性发热10d。皮肤科情况:双面颊、下颌、颈部、躯干、四肢可见多发直径约0.5 12cm大小的暗红色水肿性丘疹、扁平斑块,大部分呈圆形或椭圆形,界清,部分皮疹边缘规则隆起呈堤状,中央可见糜烂、溃疡,部分表面有黄色分泌物渗出及黑色痂皮,部分溃疡基底见颗粒状新鲜肉芽生长。皮损组织病理示:角化过度,角化不全,角层内可见中性粒细胞微脓疡,表皮不规则增生,棘层肥厚,表皮突向下延长,真皮乳头水肿,真皮内炎细胞呈结节状浸润,可见较多组织细胞样单一核细胞、中性粒细胞以及嗜酸性粒细胞,皮下脂肪层未见明显炎细胞浸润。免疫组织化学示:较多的组织细胞样单一核细胞及中性粒细胞MPO、CD68表型阳性。诊断:组织细胞型Sweet综合征。

A Case of Histiocytoid Sweet Syndrome in an Infant

A male infant at the age of 6 and a half months was referred to our department because of his 25-day history of painful edematous red plaques and vesicles on his face,neck,trunk and extremities,which had been aggravating with intermittent fever for 10 days.Dermatological examination showed multiple edematous raised flat erythematous plaques,about 0.5~12 cm in diameter,on his face,neck,trunk and extremities,with the edge raised in a levee-like shape and rupture on the surface of most lesions.Yellowish secretions and pus substance could be seen on the base of the lesions,with blood crust and black crust on the surface.Histopathology examination showed hyperkeratosis,neutrophilic microabscess at the stratum corneum,irregular epidermal hyperplasia,acanthosis,rete ridges extending downward,edema at the papillary layer,and nodular infiltration of inflammatory cells in the dermis.The infiltrated cells included many histiocytoid mononuclear cells and neutrophils,as well as some eosinophils.Immunohistochemistry examination showed that many histiocytoid mononuclear cells and neutrophils were positive for MPO and CD68.The patient was diagnosed with histiocytoid sweet syndrome.