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色素性紫癜性皮病直接免疫荧光检查结果的回顾性研究。

A retrospective study on the direct immunofluorescence findings in pigmented purpuric dermatosis.

作者信息

Ilagan Franchesca Marie D, Wu Yu-Hung

机构信息

Department of Dermatology, University of Santo Tomas Hospital, Manila, Philippines.

Department of Medicine, MacKay Medical College, New Taipei City, Taiwan.

出版信息

J Cutan Pathol. 2024 Jan;51(1):63-69. doi: 10.1111/cup.14507. Epub 2023 Aug 11.

Abstract

BACKGROUND

Pigmented purpuric dermatosis (PPD) is characterized by grouped petechiae, purpuric macules, and pigmentation in the bilateral lower extremities. It runs a chronic and relapsing course. Pathophysiology is poorly understood, but it has been proposed to be an immune-complex disease or capillaritis. This study aimed to determine the incidence and patterns of positive direct immunofluorescence (DIF) findings in patients with clinically and histopathologically confirmed PPD. The association between DIF deposition type and clinical profile was also analyzed.

METHODS

Patients with a clinical and histopathologic PPD diagnosis who had undergone DIF studies at a tertiary medical center with attached dermatopathology and immunofluorescence diagnostic centers between January 2002 and December 2021 were included in this study. Data on age, sex, disease duration, comorbidities, and drug intake were collected from medical records.

RESULTS

There were 65 patients who satisfied the inclusion criteria. Among them, 58 (89%) had at least one positive finding and 53 (82%) were vascular deposition of immunoglobulin (Ig), complement, or fibrinogen. The most common vascular deposition was fibrinogen (71%) followed by C3 (62%), IgM (18%), IgA (6%), and IgG (3%). Fibrinogen deposition was associated with hypertension (p < 0.03). There was no association between vascular DIF deposition of IgG, IgA, and C3, with age, sex, comorbidities, disease duration, and drug history.

CONCLUSION

The most common DIF findings in PPD were vascular deposition of fibrinogen and C3, with or without Ig presence. DIF findings supported a vascular origin in PPD but not an immune complex-mediated disease. Hypertension was associated with fibrinogen deposition and may play a role in its pathophysiology.

摘要

背景

色素性紫癜性皮病(PPD)的特征为双侧下肢出现成簇的瘀点、紫癜性斑疹及色素沉着。病程呈慢性且易复发。其病理生理学机制尚不清楚,但有人提出它是一种免疫复合物疾病或毛细血管炎。本研究旨在确定临床和组织病理学确诊的PPD患者中直接免疫荧光(DIF)阳性结果的发生率及模式。还分析了DIF沉积类型与临床特征之间的关联。

方法

本研究纳入了2002年1月至2021年12月期间在一家设有皮肤病理学和免疫荧光诊断中心的三级医疗中心接受DIF检查的临床及组织病理学诊断为PPD的患者。从病历中收集年龄、性别、病程、合并症及药物服用情况等数据。

结果

有65例患者符合纳入标准。其中,58例(89%)至少有一项阳性结果,53例(82%)为免疫球蛋白(Ig)、补体或纤维蛋白原的血管沉积。最常见的血管沉积是纤维蛋白原(71%),其次是C3(62%)、IgM(18%)、IgA(6%)和IgG(3%)。纤维蛋白原沉积与高血压相关(p < 0.03)。IgG、IgA和C3的血管DIF沉积与年龄、性别、合并症、病程及用药史之间无关联。

结论

PPD最常见的DIF结果是纤维蛋白原和C3的血管沉积,有无Ig存在均可。DIF结果支持PPD的血管起源而非免疫复合物介导的疾病。高血压与纤维蛋白原沉积相关,可能在其病理生理学中起作用。

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