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肉芽肿性淋巴样丘疹病 1 例报告。

A case report of granulomatous lymphomatoid papulosis.

机构信息

Department of Dermatology, College of Medicine, University of Florida, Gainesville, Florida, USA.

出版信息

Dermatol Online J. 2023 Feb 15;29(1). doi: 10.5070/D329160213.

Abstract

Lymphomatoid papulosis is a chronic CD30-positive cutaneous lymphoproliferative disorder that is characterized by recurring red-brown necrotic papules. It exhibits a wide spectrum of histopathologic findings and is often associated with cutaneous T-cell lymphomas. Six different histological subtypes have been classified by the WHO, but there is limited understanding regarding rare histopathologic variants. We describe a 51-year-old man who presented with recurring, necrotic papules for 6 years that progressed to involve the face, scalp, trunk, axilla, and scrotum. Histopathology demonstrated sarcoidal granulomas, along with a CD30-positive T cell infiltrate which demonstrated clonality by T cell receptor gamma gene rearrangement. A diagnosis of lymphomatoid papulosis associated with granulomas was established based on the clinical and histopathologic presentation. The clinical understanding of granulomatous lymphomatoid papulosis is limited in the available literature and more awareness of this histopathologic variant is required for accurate classification of this disorder.

摘要

蕈样肉芽肿病是一种慢性 CD30 阳性皮肤淋巴增生性疾病,其特征为反复发作的红棕色坏死性丘疹。它表现出广泛的组织病理学发现,并常与皮肤 T 细胞淋巴瘤相关。世界卫生组织(WHO)已经对蕈样肉芽肿病进行了六种不同的组织学分类,但对于罕见的组织病理学变异体的了解有限。我们描述了一名 51 岁男性,他因反复发作的坏死性丘疹就诊,病史长达 6 年,累及面部、头皮、躯干、腋窝和阴囊。组织病理学显示类肉瘤样肉芽肿,伴有 CD30 阳性 T 细胞浸润,通过 T 细胞受体γ基因重排显示克隆性。根据临床表现和组织病理学表现,诊断为蕈样肉芽肿病伴肉芽肿。肉芽肿性蕈样肉芽肿病的临床认识在现有文献中有限,需要提高对这种组织病理学变异体的认识,以便准确分类这种疾病。

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