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口服甲基强的松龙治疗大疱性类天疱疮后发生广泛皮肤医源性卡波西肉瘤:1例罕见的中国病例报告

Extensive cutaneous iatrogenic Kaposi's sarcoma after bullous pemphigoid treatment with oral methylprednisolone: a rare Chinese case report.

作者信息

Chen Sheng-An, Wang Lan-Ting, Chen Zi-Hua, Yang Fan-Ping, Zhu Qin-Yuan, Chen Hu-Yan, Luo Xiao-Qun

机构信息

Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China.

出版信息

J Int Med Res. 2021 Jan;49(1):300060520982829. doi: 10.1177/0300060520982829.

Abstract

Bullous pemphigoid (BP) is an autoimmune disease that requires immunosuppressive therapy. Systemic corticosteroids are considered the standard treatment for moderate-to-severe BP. Kaposi's sarcoma (KS) is a rare multifocal endothelial tumour that affects the skin, mucosa and viscera. As an angioproliferative disease of obscure aetiopathogenesis and histogenesis, KS is associated with human herpesvirus 8 (HHV-8). This current case report describes a rare occurrence of extensive cutaneous KS in a 60-year-old Chinese male patient after oral methylprednisolone treatment for BP with an emphasis on its pathological characterization. A total of more than 40 nodules were found on his trunk and lower limbs covering more than 20% of his body surface area. Immunohistochemical staining of biopsy samples from the lesion showed the patient was positive for HHV-8, CD31, CD34, XIIIa, ERG and Ki-67. The Epstein-Barr virus test showed the patient tested negative for immunoglobulin (Ig)A and IgM, but was positive for IgG. Immunosuppression associated with the treatment for BP may activate a latent HHV-8 infection and induce the development of KS.

摘要

大疱性类天疱疮(BP)是一种需要免疫抑制治疗的自身免疫性疾病。全身用糖皮质激素被认为是中重度BP的标准治疗方法。卡波西肉瘤(KS)是一种罕见的多灶性内皮肿瘤,可累及皮肤、黏膜和内脏。作为一种病因和组织发生机制不明的血管增殖性疾病,KS与人疱疹病毒8型(HHV-8)相关。本病例报告描述了一名60岁中国男性患者在口服甲泼尼龙治疗BP后罕见地出现广泛皮肤KS的情况,并着重介绍了其病理特征。在他的躯干和下肢共发现40多个结节,覆盖其体表面积的20%以上。病变活检样本的免疫组化染色显示患者HHV-8、CD31、CD34、XIIIa、ERG和Ki-67呈阳性。爱泼斯坦-巴尔病毒检测显示患者免疫球蛋白(Ig)A和IgM检测为阴性,但IgG检测为阳性。与BP治疗相关的免疫抑制可能激活潜伏的HHV-8感染并诱发KS的发生。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c894/7841242/a012ad810673/10.1177_0300060520982829-fig1.jpg

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