Paraneoplastic pemphigus presenting as a lichen planus pemphigoides-like eruption.
Paraneoplastic pemphigus presenting as a lichen planus pemphigoides-like eruption.
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DOI：
10.1001/archderm.1993.01680280054010
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发表时间：
1993-07
影响因子：
--
通讯作者：
Seth R. Stevens;C. E. Griffiths;G. J. Anhalt;K. D. Cooper
中科院分区：
文献类型：
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作者：
Seth R. Stevens;C. E. Griffiths;G. J. Anhalt;K. D. Cooper
背景副肿瘤性天疱疮是指一种特殊的疾病实体，其中瘤形成与严重的粘膜溃疡和多形性皮疹有关，有时类似于多形性红斑。标准底物上的直接免疫荧光和间接免疫荧光与寻常型天疱疮相似;然而，副肿瘤性天疱疮血清独特地结合所有上皮细胞，并具有独特的免疫沉淀特征。观察一名63岁女性慢性淋巴细胞白血病患者出现严重的皮肤粘膜水疱性大疱性皮疹，其临床和组织病理学特征为类天疱疮样扁平苔藓，演变为Stevens-Johnson样表现，免疫荧光结果为寻常型天疱疮。通过间接免疫荧光和免疫沉淀标准对她的血清进行评价，证实了副肿瘤性天疱疮特异性自身抗体的存在。结论现在必须扩大副肿瘤性天疱疮的范围，以包括临床和组织学上表现为扁平苔藓类天疱疮样皮疹的病例。
BACKGROUND Paraneoplastic pemphigus refers to a specific disease entity in which neoplasia is associated with severe mucosal ulceration and polymorphous cutaneous eruptions, sometimes resembling erythema multiforme. Direct immunofluorescence and indirect immunofluorescence on standard substrates are similar to pemphigus vulgaris; however, paraneoplastic pemphigus serum uniquely binds to all epithelia and has distinctive immunoprecipitation characteristics. OBSERVATIONS A 63-year-old woman with chronic lymphocytic leukemia developed a severe, mucocutaneous, vesiculobullous eruption with the clinical and histopathologic characteristics of lichen planus pemphigoides evolving into a Stevens-Johnson-like presentation and the immunofluorescence findings of pemphigus vulgaris. Evaluation of her serum confirmed the presence of autoantibodies specific for paraneoplastic pemphigus by indirect immunofluorescence and immunoprecipitation criteria. CONCLUSION The spectrum of paraneoplastic pemphigus must now be expanded to include cases that present clinically and histologically as a lichen planus pemphigoides-like eruption.