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双侧弥漫性葡萄膜黑色素细胞增生伴多发性弥漫性皮肤黑色素细胞增生副肿瘤综合征:病例报告。

Bilateral diffuse uveal melanocytic proliferation with multifocal diffuse integumentary melanocytic proliferation paraneoplastic syndrome: A case report.

机构信息

Department of Dermatology, Escuela de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.

Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, USA.

出版信息

Australas J Dermatol. 2021 Aug;62(3):386-389. doi: 10.1111/ajd.13617. Epub 2021 May 11.

Abstract

Bilateral diffuse uveal melanocytic proliferation (B-DUMP) is a rare paraneoplastic syndrome typically presenting with bilateral visual loss. B-DUMP is associated with extraocular systemic malignancies with the most common being lung cancer in males and uro-gynaecological cancer in females (mainly ovarian cancer). Cutaneous and/or mucosal involvement in patients with B-DUMP has been reported but it is not well characterised. Herein, we present a female in her 70s with diagnosis of stage IV vaginal clear-cell carcinoma and metastatic melanoma of unknown primary that developed progressive bilateral loss of visual acuity compatible with 'B-DUMP'. Simultaneously, she developed multifocal bilateral bluish-greyish patches on the skin that were shown to have a proliferation of dermal melanocytes. We propose that the clinical and histopathologic cutaneous findings seen in patients with B-DUMP be termed 'diffuse integumentary melanocytic proliferation (DIMP)'.

摘要

双侧弥漫性葡萄膜黑色素细胞增生症(B-DUMP)是一种罕见的副肿瘤综合征,典型表现为双侧视力丧失。B-DUMP 与眼外全身恶性肿瘤有关,最常见于男性的肺癌和女性的泌尿生殖系统癌症(主要是卵巢癌)。已有报道称 B-DUMP 患者存在皮肤和/或黏膜受累,但特征描述不详。在此,我们报告了一名 70 多岁的女性,诊断为 IV 期阴道透明细胞癌和不明原发灶的转移性黑色素瘤,出现进行性双侧视力丧失,符合“B-DUMP”表现。同时,她的皮肤出现多发性双侧蓝灰色斑块,组织病理学显示真皮黑色素细胞增生。我们提出,将 B-DUMP 患者的临床和组织病理学皮肤表现称为“弥漫性皮肤黑色素细胞增生症(DIMP)”。

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