Section of Dermatology, Department of Surgery and Translational Medicine, University of Florence, Italy.
G Ital Dermatol Venereol. 2013 Apr;148(2):163-9.
The aim of this paper to report the main clinical and immunopathological findings of our case series of 159 patients with dermatitis herpetiformis (DH).
All DH patients that were diagnosed from 1995 to 2012 at the Section of Dermatology of the University of Florence were included in the study. Clinical data were collected for each patient. Moreover, histopathological examination on both the skin and the small bowel, direct immunofluorescence on perilesional skin as well as the search for anti-endomysium and anti-tissue transglutaminsase antibodies (tTG) were performed.
A total of 159 patients with a male predominance were enrolled. About 36% of the patients were below the age of 20. The most frequent clinical features seen in our DH patients were represented by figurate erythema, wheals, papules and scratching lesions, while the knees, elbows and buttocks were the most commonly involved sites. All the 22 patients that underwent a bowel biopsy showed the typical alterations found in celiac disease. Moreover, 100% of the patients showed granular IgA deposits at the papillary tips. Finally, anti-endomysium and anti-tTG antibodies were present in 90% and 96% of the patients, respectively.
We reported one of the largest case series of patients with DH from a single center. Our study confirmed most of the data from the Literature, and in particular the association of DH to histologically proven CD in all the biopsied cases. Another interesting finding of our study is the high prevalence of DH within pediatric patients, that is usually underreported.
本文旨在报告我们的 159 例疱疹样皮炎(DH)患者的主要临床和免疫病理学发现。
本研究纳入了 1995 年至 2012 年期间在佛罗伦萨大学皮肤科确诊的所有 DH 患者。收集了每位患者的临床数据。此外,对皮肤和小肠进行了组织病理学检查,对皮损周围皮肤进行了直接免疫荧光检查,并进行了抗内肌膜和抗组织转谷氨酰胺酶抗体(tTG)检测。
共纳入了 159 例男性为主的患者。约 36%的患者年龄在 20 岁以下。我们的 DH 患者最常见的临床特征表现为图形红斑、风团、丘疹和搔抓性皮损,而膝盖、肘部和臀部是最常受累的部位。所有接受肠活检的 22 例患者均显示出与乳糜泻一致的典型改变。此外,100%的患者在乳头尖端显示颗粒状 IgA 沉积。最后,90%的患者存在抗内肌膜抗体,96%的患者存在抗 tTG 抗体。
我们报告了来自单一中心的最大 DH 患者系列之一。我们的研究证实了大多数文献数据,特别是在所有活检病例中 DH 与组织学证实的 CD 的关联。我们研究的另一个有趣发现是儿科患者中 DH 的高发率,这通常报道较少。