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“连接成人和幼儿的斑点”:过敏性紫癜(IgA 血管炎)的临床病理研究

"Joining the Spots in Adults and Young Tots": A Clinicopathological Study of Henoch-Schönlein Purpura (IgA Vasculitis).

作者信息

Kedia Priyanka P, Tirumalae Rajalakshmi, Puttegowda Divya, Antony Meryl

机构信息

Departments of *Pathology, and†Dermatology, St. John's Medical College and Hospital, Bangalore, India. Dr. Divya is now a Consultant Histopathologist, Columbia Asia Referral Hospital, Yeshwantpur, India.

出版信息

Am J Dermatopathol. 2017 Aug;39(8):587-592. doi: 10.1097/DAD.0000000000000761.

DOI:10.1097/DAD.0000000000000761
PMID:28731865
Abstract

INTRODUCTION

Henoch-Schönlein Purpura (HSP; IgA vasculitis), the most common vasculitis of childhood, has a wide spectrum of clinical manifestations ranging from palpable purpura with abdominal pain and arthritis to the more morbid renal involvement.

AIMS AND OBJECTIVES

To study and correlate the clinical presentation, laboratory values, skin and renal histopathology and immunofluoroscence findings in HSP.

MATERIAL AND METHODS

A total of 44 cases of HSP from March 2011 to February 2014 were studied for the above features along with their clinical outcomes.

RESULTS

The mean age of the patients at presentation was 28.4 years (range 4.5-69 years) with 26.2% being children younger than 15 years. Purpura at presentation was seen in all with 77.1% cases exhibiting extracutaneous involvement. Skin biopsy revealed leukocytoclastic vasculitis in all cases, dominant IgA deposition in the papillary dermal vessels in 43 cases (97.7%), associated C3 in 10 cases (23.3%), and full-house positivity in 1 case. One case without skin immunofluorescence had renal mesangial IgA deposits. Nine cases (20.9%) with an average age of 39 years had renal symptoms and displayed varied histology from focal mesangioproliferative glomerulonephritis to crescentic. All of them had dominant mesangial IgA deposits, majority falling into International Study of Kidney Disease in Children (ISKDC) class IIIb. Peritubular capillaritis was noted in 3 cases. Two patients (4.5%) progressed to end stage renal disease, one of whom had cellular crescents at presentation.

CONCLUSIONS

HSP was seen both in children and adults. Renal involvement was seen in 20.9% cases, all older than 14 years, and 66.7% with extracutaneous manifestations. The outcome in this series was favorable in 96% cases.

摘要

引言

过敏性紫癜(HSP;IgA血管炎)是儿童最常见的血管炎,临床表现广泛,从伴有腹痛和关节炎的可触及性紫癜到更严重的肾脏受累。

目的

研究过敏性紫癜的临床表现、实验室检查值、皮肤和肾脏组织病理学及免疫荧光结果,并进行相关性分析。

材料与方法

对2011年3月至2014年2月期间的44例过敏性紫癜患者进行上述特征及临床结局的研究。

结果

患者就诊时的平均年龄为28.4岁(范围4.5 - 69岁),其中26.2%为15岁以下儿童。所有患者均出现紫癜,77.1%的病例有皮肤外受累表现。皮肤活检显示所有病例均有白细胞破碎性血管炎,43例(97.7%)在乳头真皮血管中有主要IgA沉积,10例(23.3%)伴有C3沉积,1例呈满堂阳性。1例未进行皮肤免疫荧光检查的患者有肾系膜IgA沉积。9例(20.9%)平均年龄为39岁的患者有肾脏症状,组织学表现多样,从局灶性系膜增生性肾小球肾炎到新月体性肾炎。所有患者均有主要的系膜IgA沉积,大多数属于儿童肾脏病国际研究(ISKDC)IIIb级。3例患者出现肾小管周围毛细血管炎。2例患者(4.5%)进展为终末期肾病,其中1例就诊时即有细胞性新月体。

结论

过敏性紫癜在儿童和成人中均有发生。20.9%的病例出现肾脏受累,均为14岁以上患者,66.7%的病例有皮肤外表现。本系列病例中96%的患者预后良好。

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