伴发滤泡树突状细胞肉瘤的副肿瘤天疱疮1例报告

Paraneoplastic Pemphigus Association with Foliculai Dendritic Cell Sarcoma

讨论成功救治的1例滤泡树突状细胞肉瘤伴发的副肿瘤天疱疮患者。女,56岁,因口腔溃疡1个月,全身皮疹10天入院。临床表现为口唇溃疡,躯干、四肢扁平苔藓样和多形红斑样皮疹,皮损组织病理见表皮内泡,孤立坏死角质形成细胞,基底细胞空泡化和真皮大量淋巴细胞浸润等副肿瘤天疱疮表现。患者血清行鼠膀胱为底物的IIF为阳性,表皮浸出物为底物免疫印迹试验发现190、210 KD阳性条带。PET/CT检查示腹膜后肿物,彻底手术切除并经组织病理证实为滤泡树突状细胞肉瘤。按照我国自行总结的治疗方案,术前、术中及术后均给予静脉点滴免疫球蛋白,给予糖皮质激素治疗,手术后患者皮疹逐渐好转,术后两个月时复查躯干四肢皮损基本消退,术后半年口腔溃疡大部分愈合,无皮疹复发。

To discuss a case with paraneoplastic pemphigus association with follicular dendritic cell sarcoma and a satisfactory response to treatment has been reached. The patient was a 56 - year - old female who have had oral and lips ulcer for 1 month and skin rashs ware occurred all over the body for 10 days. She presented with recalcitrant erosions of oral cavity, tongue and genital mucosa, followed by hyperemic conjunctivitis and polymorphous eruptions resembling the erythema multiform and lichen planus on trunk and distal extremities. Histological examination of the cuta- neous lesions demonstrated epidermal acantholysis and intraepidermal acantholytic blisters, necro- sis of keratinocyte, interface vacuolar degeneration and diffuse lymphocyte infiltration in dermis. Indirect immunofluorescence on rat bladder revealed the deposition of IgG from the patients＇ serum in intercellular spaces of the epithelia. Western blot demonstrated antiepidermal antibodies, 210 kDa envoplakin and 190 kDa periplakin were positive. PET - CT scan revealed a mass in her ret- roperitoneal region. After the total surgical removal of the tumor, pathology and immunohistochem- istry examination further approved the diagnosis of follicular dendritic cell sarcoma. The patient was treated according to the clinical criteria for management of the PNP patients. The skin lesion improved in 2 months and the oral lesion recovered in 6 months after the operation. No recurrent tumor and skin lesion wete found during the 6 months follow up.