Castleman病继发副肿瘤性天疱疮和系统性红斑狼疮一例

Castleman＇s disease with secondary paraneoplastic pemphigus and systemic lupus erythematosus： a case report

患者男,47岁.反复口腔溃疡1年余,全身水疱9个月余.体检：口腔内见多处溃疡面,唇黏膜见片状糜烂面、黄色或黑褐色痂皮和嘴角浸渍发白,张口困难;鼻孔黏膜交界处、眼睑缘和阴茎冠状沟见黄褐色痂和脱痂后的淡红斑;躯干、四肢见散在红斑和水疱,大疱疱壁紧贴皮肤、部分破裂,基底潮红,类似多形红斑样改变,部分疱壁浑浊,见白色药痂黏着或黑色痂皮,尼氏征阴性;10指（趾）甲周见皱缩的水肿性暗红斑.水疱组织病理和皮损直接免疫荧光检查符合天疱疮诊断.纵隔肿瘤组织病理和免疫组化符合Castleman病（透明血管型）伴滤泡树突细胞增生.实验室检查：血清补体C3下降以及抗核抗体、抗核小体抗体、dsDNA抗体均阳性.诊断：Casfleman病、副肿瘤性天疱疮、系统性红斑狼疮.用小剂量泼尼松和硫唑嘌呤治疗2个月后皮疹完全消退,免疫抑制药物缓慢减量,治疗6个月后,患者自行停用免疫抑制药物改为中药治疗,皮疹发展为中毒性表皮坏死松解症,最后死亡.该病在肿瘤切除后免疫抑制药物需维持一段较长时间,否则可导致皮疹反复或加重.

A 47-year-old man presented with recurrent oral ulcerations for more than 1 year and generalized vesicles for 9 months.Physical examination revealed multiple oral ulcers,patchy labial erosions with yellow or dark brown crusts,maceration and blanching of the angle of mouth resulting in difficulty in mouth opening.Cinnamomeous crusts were seen on the nasal limen,palpebral margin and coronary sulcus of penis,with pale red macules after decrustation.Diffuse erythematous patches and bullae were present on the trunk and extremities.The walls of bullae tightly clung to the skin,and some bullae were ruptured,leaving an erythematous and moist surface and giving an erythema multiforme-like appearance; some bullae were turbid and covered with adherent drugcontaining white crusts or black crusts.Nikolsky＇s sign was negative.Shrinking,edematous dark-red patches were seen in periungual regions of all the fingers and toes.Histologic biopsy and direct immunofluorescence examination of bullous lesions confirmed a diagnosis of paraneoplastic pemphigus （PNP）.Histopathological and immunohistochemical findings from the mediastinal tumor were consistent with Castleman＇s disease （hyalinevascular type） with proliferation of follicular dendritic cells.Laboratory examination revealed a decrease in serum complement C3 and the presence of antinuclear,anti-nucleosome and anti-dsDNA antibodies.The final diagnosis included Castleman＇s disease,PNP and systemic lupus erythematosus.After 2 months of treatment with low-dose prednisone and azathioprine,skin lesions completely regressed.Then,the dose of prednisone and azathioprine was tapered.Six months later,the patient himself suddenly withdrew prednisone and azathioprine,and began to take traditional Chinese medicine; thereafter,the lesions developed into toxic epidermal necrolysis （TEN）,and the patient died finally.This case demonstrates that immunosuppressive therapy should be maintained for a long period of time in patients with Castleman＇s disease after tumor removal,otherwise,skin lesions may recur or get worse.