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妊娠类天疱疮:伊朗西南部的一项回顾性研究。

Pemphigoid gestationis: a retrospective study in southwest Iran.

作者信息

Rassai Sima, Pazyar Nader, Alavi Seyed Milad, Kazerouni Afshin, Feily Amir

机构信息

Department of Dermatology, Jundishapur University of Medical Sciences, Ahvaz, Iran.

出版信息

Acta Med Iran. 2013 Jul 13;51(6):408-10.

Abstract

Pemphigoid gestationis (PG) is a rare autoimmune bullous dermatosis of pregnancy usually presents in the second or third trimester. It is characterized by pruritic, urticarial plaques with the development of tense vesicles and bullae within the lesions. Pathogenesis of PG is not fully established, however, most patients develop circulating autoantibodies targeting the bullous pemphigoid (BP) 180 antigen. The aim of this work is to draw a profile of the epidemiology, clinical aspects, treatment and evolution of the disease by studying hospital series. We retrospectively investigated the 13 patients who were diagnosed with PG based on hospital data at the Referral Center of Southwest Iran located in Ahvaz city between March 2002 and March 2011. The age of onset was 21 to 40 years (mean age: 27.5 years). The onset of the disease occurred in the second trimester of pregnancy in 6 patients and in the third trimester of pregnancy in 4 patients. One patient had a flare up of disease during the first trimester and two out of cases in puerperium period. In all cases, pruritus was the first symptom, followed by an erythematous vesiculobullous eruption.  The diagnosis of PG was confirmed by skin biopsy. Ten out of the patients treated with oral corticosteroids (0.5-1 mg/kg/day), one of the patients underwent oral corticosteroids plus topical glucocorticoid and the last patient treated with topical glucocorticoid. PG remains a rare dermatosis of pregnancy. Our series had two particularities compared to other studies: high frequency in primigravida and the frequent involvement of the face. Additionally our study demonstrated that improvement could occur faster and provide acceptable management if the treatment of the patients would be implemented sooner.

摘要

妊娠类天疱疮(PG)是一种罕见的妊娠自身免疫性大疱性皮肤病,通常发生在妊娠中期或晚期。其特征为瘙痒性荨麻疹斑块,皮损内出现紧张性水疱和大疱。PG的发病机制尚未完全明确,然而,大多数患者会产生针对大疱性类天疱疮(BP)180抗原的循环自身抗体。这项研究的目的是通过研究医院病例系列来描绘该疾病的流行病学、临床特征、治疗及转归情况。我们回顾性调查了2002年3月至2011年3月期间在位于阿瓦士市的伊朗西南部转诊中心根据医院数据被诊断为PG的13例患者。发病年龄为21至40岁(平均年龄:27.5岁)。6例患者疾病发作于妊娠中期,4例患者发作于妊娠晚期。1例患者在妊娠早期病情复发,2例在产褥期发病。所有病例中,瘙痒是首发症状,随后出现红斑性水疱大疱性皮疹。PG的诊断通过皮肤活检得以证实。10例患者接受口服糖皮质激素治疗(0.5 - 1mg/kg/天),1例患者接受口服糖皮质激素加外用糖皮质激素治疗,最后1例患者仅接受外用糖皮质激素治疗。PG仍然是一种罕见的妊娠皮肤病。与其他研究相比,我们的病例系列有两个特点:初产妇发病率高以及面部频繁受累。此外,我们的研究表明,如果能更早地对患者进行治疗,病情改善可能会更快且能提供可接受的治疗效果。

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