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[疱疹样皮炎患者临床、组织学及免疫学特征的回顾性研究。西班牙巴塞罗那临床医院1995年至2010年的经验及文献综述]

[A retrospective study of clinical, histological, and immunological characteristics in patients with dermatitis herpetiformis. The experience of Hospital Clinic de Barcelona, Spain, between 1995 and 2010 and a review of the literature].

作者信息

Fuertes I, Mascaró J M, Bombí J A, Iranzo P

机构信息

Servicio de Dermatología, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain.

出版信息

Actas Dermosifiliogr. 2011 Nov;102(9):699-705. doi: 10.1016/j.ad.2010.11.007. Epub 2011 Mar 4.

Abstract

BACKGROUND AND OBJECTIVES

Dermatitis herpetiformis is a chronic bullous disease that is currently considered a cutaneous expression of gluten hypersensitivity. The aim of this study was to analyze and describe the clinical, histological, and immunopathological characteristics of patients with dermatitis herpetiformis assessed at Hospital Clinic de Barcelona, Spain between 1995 and 2010.

MATERIAL AND METHODS

Demographic, clinical, serologic, and histopathological data were reviewed for 33 patients with dermatitis herpetiformis.

RESULTS

The median age of the patients at the time of disease onset was 30 years and the majority were men. Associated autoimmune disease was present in 49% of patients. In 6 patients, celiac disease was diagnosed before dermatitis herpetiformis. Although excoriations were the most predominant lesions, 9 patients had blisters. Histological findings in skin lesions were compatible with dermatitis herpetiformis in 46% of cases. The most frequently observed staining pattern by indirect immunofluorescence was the presence of granular immunoglobulin A deposits in the basement membrane (62%). More than 80% of intestinal biopsies were compatible with celiac disease. Antibodies linked to gluten sensitivity were observed in 79% of patients. Only 1 malignant tumor was detected.

CONCLUSIONS

Notable findings were the frequent presence of bullous lesions, the high prevalence of celiac disease, and the positive findings on intestinal biopsy, all of which are suggestive of late diagnosis. Our findings confirm the lack of specificity of conventional histology in dermatitis herpetiformis and the association of the disease with other immunological disorders.

摘要

背景与目的

疱疹样皮炎是一种慢性大疱性疾病,目前被认为是麸质过敏的皮肤表现。本研究的目的是分析和描述1995年至2010年期间在西班牙巴塞罗那临床医院接受评估的疱疹样皮炎患者的临床、组织学和免疫病理学特征。

材料与方法

回顾了33例疱疹样皮炎患者的人口统计学、临床、血清学和组织病理学数据。

结果

患者发病时的中位年龄为30岁,大多数为男性。49%的患者存在相关自身免疫性疾病。6例患者在疱疹样皮炎之前被诊断为乳糜泻。虽然擦伤是最主要的皮损,但9例患者有水疱。46%的皮肤病变组织学表现符合疱疹样皮炎。间接免疫荧光最常观察到的染色模式是基底膜中有颗粒状免疫球蛋白A沉积(62%)。超过80%的肠道活检符合乳糜泻。79%的患者观察到与麸质敏感性相关的抗体。仅检测到1例恶性肿瘤。

结论

显著的发现是水疱性皮损频繁出现、乳糜泻的高患病率以及肠道活检的阳性结果,所有这些都提示诊断较晚。我们的发现证实了传统组织学在疱疹样皮炎中的非特异性以及该疾病与其他免疫性疾病的关联。

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