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1例具有增殖性天疱疮特征的外阴棘层松解性皮肤病。

A case of acantholytic dermatosis of the vulva with features of pemphigus vegetans.

作者信息

Wong K T, Wong K K

机构信息

Department of Pathology, Faculty of Medicine, University of Malaya, Kuala Lumpur.

出版信息

J Cutan Pathol. 1994 Oct;21(5):453-6. doi: 10.1111/j.1600-0560.1994.tb00288.x.

DOI:10.1111/j.1600-0560.1994.tb00288.x
PMID:7868757
Abstract

We report an unusual case of vulvar acantholytic dermatosis with features of pemphigus vegetans in a 22-year-old Indian girl who presented with a "warty" lesion in her left labium majus. Following excision of this lesion, she presented with 2 localized recurrent lesions on the left and right labia majora about 2 1/2 years later which were also excised. All 3 biopsies showed histological features typical of pemphigus which included extensive suprabasal acantholysis with bullae formation, prominent villus-like processes at the base of the bullae, focal hyperkeratosis and papillomatosis, and the occasional mixed neutrophil and eosinophilic intraepidermal abscess. IgG and C3 immunofluorescence was positive in the intercellular spaces of the epidermis. These lesions, which probably represent a form of pemphigus vegetans, have not been previously reported as a cause of localized vulvar acantholytic dermatosis.

摘要

我们报告了一例罕见的外阴棘层松解性皮肤病病例,该病例具有增殖性天疱疮的特征,患者为一名22岁的印度女孩,其左大阴唇出现“疣状”病变。切除该病变后,约2年半后她在左右大阴唇又出现了2处局限性复发性病变,这些病变也被切除。所有3次活检均显示出天疱疮典型的组织学特征,包括广泛的基底层上棘层松解伴水疱形成、水疱底部突出的绒毛状突起、局灶性角化过度和乳头瘤样增生,以及偶尔出现的混合性中性粒细胞和嗜酸性粒细胞表皮内脓肿。IgG和C3免疫荧光在表皮细胞间隙呈阳性。这些病变可能代表增殖性天疱疮的一种形式,此前尚未报道其为局限性外阴棘层松解性皮肤病的病因。

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