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硼替佐米和地塞米松治疗后伴单克隆丙种球蛋白血症的播散性坏疽性脓皮病戏剧性缓解。

Dramatic resolution of disseminated pyoderma gangrenosum associated with monoclonal gammopathy after therapy with bortezomib and dexamethasone.

机构信息

Department of Dermatology, Hospital 12 de Octubre, Madrid, Spain.

Department of Hematology, Hospital 12 de Octubre, Madrid, Spain.

出版信息

Int Wound J. 2017 Dec;14(6):1382-1384. doi: 10.1111/iwj.12746. Epub 2017 Mar 29.

Abstract

Pyoderma gangrenosum (PG) is an uncommon inflammatory and ulcerative skin disorder, which is commonly associated with systemic conditions such as inflammatory bowel disease, arthritis and haematological malignancies. It is widely stated that control of the underlying diseases may lead to resolution of PG. However, standard of care dictates that patients suffering with monoclonal gammopathy of undetermined significance or smouldering multiple myeloma (MM) should not receive therapy unless they progress to symptomatic MM. Here, we report a woman in her 40s with a disseminated corticodependent PG, resistant to any treatment attempted, including anti-tumoral necrosis factor (TNF) therapy in which bortezomib-dexamethasone regimen results in dramatic healing of all lesions in only a month. This case supports the belief that treatment of the underlying monoclonal gammopathy could be necessary when PG presents as an aggressive, non-responding skin disease.

摘要

坏疽性脓皮病(PG)是一种罕见的炎症性和溃疡性皮肤疾病,通常与炎症性肠病、关节炎和血液系统恶性肿瘤等系统性疾病相关。广泛认为,控制基础疾病可能导致 PG 的消退。然而,标准治疗规定,患有意义未明的单克隆丙种球蛋白血症或冒烟型多发性骨髓瘤(MM)的患者不应接受治疗,除非他们进展为有症状的 MM。在这里,我们报告了一名 40 多岁的女性患有播散性皮质依赖性 PG,对任何尝试的治疗均有耐药性,包括抗肿瘤坏死因子(TNF)治疗,其中硼替佐米-地塞米松方案仅在一个月内导致所有病变明显愈合。该病例支持这样一种信念,即当 PG 表现为一种侵袭性、无反应性皮肤病时,治疗潜在的单克隆丙种球蛋白血症可能是必要的。

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