Preclaro Ivan Arni C, Wu Yu-Hung
Department of Dermatology, MacKay Memorial Hospital, Taipei 10449, Taiwan.
Department of Medicine, MacKay Medical College, New Taipei City 25245, Taiwan.
Dermatopathology (Basel). 2022 May 20;9(2):172-182. doi: 10.3390/dermatopathology9020022.
Pemphigus is a chronic blistering disorder caused by autoantibodies that target desmosomal proteins in the epidermis. Acantholysis may be absent, and pemphigus may present only with spongiosis and vesiculation, thereby leading to a misdiagnosis of eczema. Herein, we conducted a retrospective, observational, single-center study to establish a pattern of spongiosis in cases of pemphigus confirmed by direct immunofluorescence. Immunopathologically diagnosed pemphigus specimens from 2001 to 2020 were retrieved, and specimens with spongiosis were analyzed for the following features: vesiculation, acantholysis, spongiosis, inflammatory cells in the epidermis, and inflammation in the dermis. Cases of spongiotic dermatitis were used as control. Out of 99 immunopathologically diagnosed pemphigus specimens, 41 samples with spongiosis were identified. About one quarter of the specimens did not have acantholysis. Spongiosis in the middle to lower thirds of the perilesional epidermis (p = 0.030), exocytosis with either neutrophils or eosinophils (p = 0.016), dermal infiltrates composed of lymphocytes, eosinophils, and neutrophils (p = 0.012), and absence of Langerhans cell microabscesses (p < 0.001) were more common in pemphigus than control. Spongiosis in pemphigus may mimic eczema in patients without acantholysis. The subtle histological findings in this study provide diagnostic clues and suggest that further immunofluorescence should be performed to confirm pemphigus diagnosis.
天疱疮是一种由自身抗体引起的慢性水疱性疾病,这些自身抗体靶向表皮中的桥粒蛋白。可能不存在棘层松解,天疱疮可能仅表现为海绵形成和水疱形成,从而导致湿疹的误诊。在此,我们进行了一项回顾性、观察性、单中心研究,以确立经直接免疫荧光证实的天疱疮病例中的海绵形成模式。检索了2001年至2020年免疫病理诊断的天疱疮标本,对有海绵形成的标本分析以下特征:水疱形成、棘层松解、海绵形成、表皮中的炎症细胞以及真皮中的炎症。海绵状皮炎病例用作对照。在99例免疫病理诊断的天疱疮标本中,鉴定出41例有海绵形成的样本。约四分之一的标本没有棘层松解。与对照相比,天疱疮患者病损周围表皮中三分之二至三分之一处的海绵形成(p = 0.030)、中性粒细胞或嗜酸性粒细胞的胞吐作用(p = 0.016)、由淋巴细胞、嗜酸性粒细胞和中性粒细胞组成的真皮浸润(p = 0.012)以及不存在朗格汉斯细胞微脓肿(p < 0.001)更为常见。在没有棘层松解的患者中,天疱疮中的海绵形成可能类似湿疹。本研究中细微的组织学发现提供了诊断线索,并表明应进一步进行免疫荧光以确诊天疱疮。