不典型药物超敏反应综合征1例

A Case of Atypical Drug Hypersensitivity Syndrome

患者女,53岁。全身皮肤红斑、瘙痒7个月,加重5d。查体见面部、颈部、躯干、四肢弥漫性潮红和肿胀,表面干燥,覆细小鳞屑,散在扁豆至分币大小糜烂面和薄壁水疱、大疱,疱液清,尼氏征阳性,外阴黏膜弥漫性糜烂渗出。皮损组织病理示表皮轻度萎缩,表皮与真皮间见水疱;真皮浅层血管扩张、充血,大量单一核细胞浸润。免疫病理示Ig A,Ig G,Ig M,C3,C4均阴性。诊断:不典型性药物超敏反应综合征。予以激素、抗过敏等治疗无效死亡。

A 53-year-old woman with erythema on entire body,felt itching for seven month. Her illness became more serious for five days. Physical examination showed that there are diffuse erythema with dry tiny scale on her face,neck,trunk and extremities. Erosion,vesicle,bulla are found on the body. The blister fluid is clearly. Nikolsky sign is positive. Genital mucosa is diffuse erosion and exudation. Histopathology by lesion biopsy showed that epidermis is mild hypokaratosis. Clear vacuoles is seen between epidermis and dermis. Dilated and congestive vascular and monocytes space in superficial dermis. Immunopathology showed that IgA,IgG,IgM,C3 are negative. The diagnosis is atypical drug hypersensitivity syndrome. The patient was dead after treatment with glucocorticoid and antihistamine.