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结节性色素性紫癜性皮病:9 例临床和组织病理学观察。

Granulomatous pigmented purpuric dermatosis: Clinical and histopathologic findings in a series of nine cases.

机构信息

Kempf und Pfaltz Histologische Diagnostik, Zurich, Switzerland.

Department of Dermatology, University Hospital Zurich, University Zurich, Zurich, Switzerland.

出版信息

J Cutan Pathol. 2022 Jul;49(7):597-603. doi: 10.1111/cup.14224. Epub 2022 Mar 22.

DOI:10.1111/cup.14224
PMID:35288961
Abstract

We report the largest case series to date of granulomatous pigmented purpuric dermatosis (GPPD), a rare variant of pigmented purpuric dermatoses (PPD). GPPD can cause diagnostic difficulties as it can be mistaken clinically and histopathologically with numerous inflammatory and infectious dermatoses or even cutaneous T-cell lymphoma. We compared the histopathological findings of nine cases of GPPD with a control group consisting of 10 randomly selected PPD of other subtypes. GPPD seems to predominantly affect the lower extremities of adult male patients; a clear association with hyperlipidemia or other systemic conditions could not be confirmed. Histopathologically, GPPD is characterized by a dermal histiocyte-rich interstitial infiltrate with or without granuloma formation, thickened capillaries, extravasated erythrocytes, and/or hemosiderin deposits. In contrast to other forms of PPD, the inflammatory infiltrate of GPPD can extend to the mid or deep dermis and the admixed lymphocytic infiltrate is mainly composed of CD8+ T-cells.

摘要

我们报告了迄今为止最大的一组肉芽肿性色素性紫癜性皮肤病(GPPD)病例系列,这是一种罕见的色素性紫癜性皮肤病(PPD)变异型。GPPD 可能会引起诊断困难,因为它在临床上和组织病理学上可能与许多炎症性和传染性皮肤病甚至皮肤 T 细胞淋巴瘤相混淆。我们将 9 例 GPPD 的组织病理学发现与由 10 例随机选择的其他亚型 PPD 组成的对照组进行了比较。GPPD 似乎主要影响成年男性患者的下肢;不能明确证实与高脂血症或其他全身情况有关。组织病理学上,GPPD 的特征是真皮组织细胞丰富的间质浸润,伴有或不伴有肉芽肿形成、毛细血管增厚、红细胞外渗和/或含铁血黄素沉积。与其他形式的 PPD 不同,GPPD 的炎症浸润可延伸至真皮中部或深部,混合性淋巴细胞浸润主要由 CD8+T 细胞组成。

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引用本文的文献

1
Granulomatous Pigmented Purpuric Dermatosis in a Patient with (Inactive) Myasthenia Gravis: A Case Report and Review of the Literature.一名(非活动期)重症肌无力患者的肉芽肿性色素性紫癜性皮病:病例报告及文献复习
Case Rep Dermatol. 2023 May 25;15(1):71-84. doi: 10.1159/000530034. eCollection 2023 Jan-Dec.