Fried R, Lynfield Y, Vitale P, Anhalt G
Dermatology Service, Department of Veterans Affairs Medical Center, Brooklyn, NY 11209.
J Am Acad Dermatol. 1993 Nov;29(5 Pt 2):815-7. doi: 10.1016/0190-9622(93)70244-n.
Paraneoplastic pemphigus is a recently described disease in which patients have polymorphous skin lesions suggestive of both erythema multiforme major and pemphigus vulgaris in association with internal neoplasms, especially non-Hodgkin's lymphoma. These patients have characteristic autoantibodies that bind specific epidermal proteins. A Nikolsky-negative bullous pemphigoid-like eruption developed within the radiation therapy field in a 72-year-old man receiving palliative treatment for recurrent large cell lymphoma. The eruption rapidly progressed to a Nikolsky-positive bullous process more typical of pemphigus vulgaris with extensive involvement of respiratory epithelia. Despite aggressive treatment with high-dose corticosteroids and antibiotics, the patient rapidly succumbed. Results of immunofluorescence studies and autopsy findings confirmed the diagnosis of paraneoplastic pemphigus.
副肿瘤性天疱疮是一种最近才被描述的疾病,患者有多种皮肤损害,提示为重症多形红斑和寻常型天疱疮,且与体内肿瘤尤其是非霍奇金淋巴瘤相关。这些患者具有结合特定表皮蛋白的特征性自身抗体。一名72岁接受复发性大细胞淋巴瘤姑息治疗的男性,在放疗区域内出现了尼氏征阴性的类大疱性类天疱疮样皮疹。皮疹迅速进展为尼氏征阳性的大疱性过程,更符合寻常型天疱疮,呼吸道上皮广泛受累。尽管用大剂量皮质类固醇和抗生素积极治疗,患者仍很快死亡。免疫荧光研究结果和尸检发现证实了副肿瘤性天疱疮的诊断。