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详细分析住院患者复杂紫癜的分布、形态和组织病理学:68 例病例系列。

A detailed analysis of the distribution, morphology, and histopathology of complex purpura in hospitalized patients: A case series of 68 patients.

机构信息

Yale School of Medicine Department of Dermatology, New Haven, Connecticut.

Mayo Clinic Department of Dermatology, Rochester, Minnesota.

出版信息

J Am Acad Dermatol. 2021 Apr;84(4):1188-1196. doi: 10.1016/j.jaad.2020.04.149. Epub 2020 May 4.

DOI:10.1016/j.jaad.2020.04.149
PMID:32376433
Abstract

BACKGROUND

Purpura in inpatients commonly leads to dermatologic consultation. The differential diagnosis is broad and algorithms are intricate.

OBJECTIVE

We evaluated inpatient consultations for complex purpura to document the most common diagnoses and to validate the true diagnostic utility of histopathology, clinical morphology, and distribution.

METHODS

We reviewed a case series of 68 inpatients during a 4-year period with a dermatologic consultation for purpura and biopsy findings of vasculitis or microvascular occlusion.

RESULTS

Key features of complex purpura are nonbranching (round) versus branching (retiform) morphology, dependent versus acral or generalized distribution, and leukocytoclastic vasculitis versus microvascular occlusion (with emphasis on depth of involvement). Dependent nonbranching purpura with only superficial vessels involved by leukocytoclastic vasculitis was most often due to IgA vasculitis or cutaneous single-organ small-vessel vasculitis. In contrast, deeper involvement by leukocytoclastic vasculitis was suggestive of systemic disease (eg, antineutrophil cytoplasmic antibody-associated vasculitis). Branching purpura was concerning, with greater than 90% sensitivity and specificity for microvascular occlusion and associated high mortality (≈50%). The majority of patients who died had acral branching lesions.

LIMITATIONS

Small sample size, inpatients at a tertiary care center, and retrospective nature are some limitations.

CONCLUSION

Nonbranching dependent purpura corresponded to leukocytoclastic vasculitis, with the most common diagnoses being IgA vasculitis or skin-limited small-vessel vasculitis; patients with deep involvement often had systemic diseases. In this series, branching purpura was due to microvascular occlusion rather than medium-vessel vasculitis, and had associated high mortality.

摘要

背景

住院患者的紫癜常导致皮肤科会诊。鉴别诊断范围广泛,算法复杂。

目的

我们评估了复杂紫癜的住院患者咨询,以记录最常见的诊断,并验证组织病理学、临床形态和分布的真正诊断效用。

方法

我们回顾了 4 年来因紫癜和血管炎或微血管阻塞的活检结果而进行皮肤科咨询的 68 例住院患者的病例系列。

结果

复杂紫癜的关键特征是非分支(圆形)与分支(网状)形态、依赖与肢端或全身性分布、白细胞碎裂性血管炎与微血管阻塞(重点是受累深度)。仅浅层血管受累的依赖非分支紫癜,伴有白细胞碎裂性血管炎,最常由 IgA 血管炎或皮肤单一器官小血管血管炎引起。相比之下,白细胞碎裂性血管炎的深部受累提示系统性疾病(例如抗中性粒细胞胞质抗体相关性血管炎)。分支性紫癜令人担忧,微血管阻塞的敏感性和特异性均大于 90%,且死亡率高(约 50%)。大多数死亡患者都有肢端分支性病变。

局限性

样本量小、三级护理中心的住院患者和回顾性研究是一些局限性。

结论

非分支性依赖性紫癜与白细胞碎裂性血管炎相对应,最常见的诊断为 IgA 血管炎或皮肤局限性小血管血管炎;深部受累的患者常有系统性疾病。在本系列中,分支性紫癜是由微血管阻塞引起,而非中血管炎,且死亡率高。

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