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线状IgA大疱性皮肤病中的火焰状图形:一项新的组织病理学发现。

Flame figures in linear IgA bullous dermatosis: a novel histopathologic finding.

作者信息

Fulton E, Jan F, Zimarowski M J

机构信息

Beth Israel Deaconess Medical Center, Department of Pathology, Harvard Medical School, Boston, Massachusetts.

出版信息

Dermatol Online J. 2017 Nov 15;23(11):13030/qt1qs7m39f.

Abstract

BACKGROUND

Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease usually with a neutrophil rich inflammatory infiltrate, and characterized by linear IgA deposition at the basement membrane zone (BMZ), and neutrophil predominant dermal inflammation. We report a case of LABD with numerous eosinophils and flame figure formation, a unique histopathologic finding not previously reported. A 69-year-old woman presented with a rapidly progressive, intensely pruritic rash over forearms, breasts, axillae, hips, and thighs. Thelesions were comprised of annular vesicles and bullae with hemorrhagic crusts and erosions. The clinical differential diagnosis included bullous pemphigoid(BP), LABD, and epidermolysis bullosa aquisita (EBA).

RESULTS

A biopsy from a bullous plaque on the wrist revealed a subepidermal blister with neutrophils and numerous eosinophils with flame figure formation.Direct immunofluorescent (DIF) microscopy revealed linear deposition of IgA at the BMZ.

CONCLUSIONS

Although unusual, the combined findings supported a diagnosis of LABD. Increased eosinophils may be associated with drug-induced LABD and may explain the numerous eosinophils in our case. It is important to be aware of this finding as the pathology may easily be misdiagnosed as BP, or possibly bullousWells syndrome. This case emphasizes that combined clinical, pathologic, and DIF findings are essential in the diagnosis of bullous dermatoses.

摘要

背景

线状IgA大疱性皮病(LABD)是一种自身免疫性表皮下大疱性疾病,通常有富含中性粒细胞的炎症浸润,其特征为在基底膜带(BMZ)有线状IgA沉积以及以中性粒细胞为主的真皮炎症。我们报告一例LABD伴有大量嗜酸性粒细胞和火焰状图形形成,这是一种以前未报道过的独特组织病理学发现。一名69岁女性患者,其前臂、乳房、腋窝、臀部和大腿出现快速进展的剧烈瘙痒性皮疹。皮损由环形水疱和大疱组成,伴有出血性结痂和糜烂。临床鉴别诊断包括大疱性类天疱疮(BP)、LABD和获得性大疱性表皮松解症(EBA)。

结果

腕部大疱性斑块活检显示表皮下大疱,有中性粒细胞和大量嗜酸性粒细胞以及火焰状图形形成。直接免疫荧光(DIF)显微镜检查显示IgA在BMZ呈线状沉积。

结论

尽管不常见,但综合这些发现支持LABD的诊断。嗜酸性粒细胞增多可能与药物诱导的LABD有关,这可能解释了我们病例中出现大量嗜酸性粒细胞的原因。认识到这一发现很重要,因为其病理可能很容易被误诊为BP,或者可能误诊为大疱性威尔斯综合征。该病例强调,临床、病理和DIF的综合发现对于大疱性皮肤病的诊断至关重要。

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