Paraneoplastic pemphigus in association with a retroperitoneal Castleman's disease presenting with a lichen planus pemphigoides‐like eruption. A case report and review of literature
Paraneoplastic pemphigus in association with a retroperitoneal Castleman's disease presenting with a lichen planus pemphigoides‐like eruption. A case report and review of literature
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副肿瘤性天疱疮与腹膜后 Castleman 病相关，表现为类天疱疮样扁平苔藓皮疹。病例报告和文献综述。
DOI：
10.1046/j.1365-2133.2001.04030.x
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发表时间：
2001
影响因子：
10.3
通讯作者：
W. Hsieh
中科院分区：
文献类型：
--
作者：
C. Hsiao;M. Hsu;J.Y.‐Y. Lee;W‐C. Chen;W. Hsieh
一名50岁男性患者出现嘴唇、口腔和肛周区域严重粘膜糜烂，躯干和四肢扁平苔藓样皮疹，手掌和脚掌鳞状斑块。临床表现为Stevens-Johnson综合征或副肿瘤性天疱疮（PNP）。组织学检查显示空泡界面和苔藓样皮炎伴角化不良和基底上棘层松解性水疱。直接免疫荧光显示IgG沉积在细胞间隙，C3沿着基底膜区呈线性沉积。间接免疫荧光显示循环IgG与细胞间染色的大鼠膀胱上皮。蛋白质印迹法显示250-和230-kDa抗原条带。其临床、组织学及免疫学特征均符合PNP的类天疱疮样扁平苔藓变异型。检测并切除腹膜后透明血管Castleman病。皮肤病变恶化后，肿瘤切除，但逐渐改善，环孢菌素和霉酚酸酯治疗后留下广泛的黑变病。
A 50‐year‐old man presented with severe mucosal erosions of the lips, oral cavity and perianal area, a lichen planus‐like eruption on the trunk and extremities and scaly plaques of the palms and soles. The clinical impression was of Stevens–Johnson syndrome, or paraneoplastic pemphigus (PNP). Histopathology revealed vacuolar interface and lichenoid dermatitis with dyskeratosis and suprabasal acantholytic vesiculation. Direct immunofluorescence showed deposition of IgG in the intercellular space and linear deposition of C3 along the basal membrane zone. Indirect immunofluorescence revealed circulating IgG with intercellular staining of the epithelium of rat urinary bladder. Western blotting demonstrated bands of 250‐ and 230‐kDa antigens. The clinical, histological and immunological features were consistent with the lichen planus pemphigoides variant of PNP. A retroperitoneal hyaline‐vascular Castleman's disease was detected and excised. The skin lesions worsened initially after tumour resection but improved gradually, leaving extensive melanosis after cyclosporin and mycophenolate mofetil treatment.
影响因子：
--
作者：
Seth R. Stevens;C. E. Griffiths;G. J. Anhalt;K. D. Cooper
通讯作者：
Seth R. Stevens;C. E. Griffiths;G. J. Anhalt;K. D. Cooper