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一例具有独特聚集表现的生殖器毛囊皮脂腺囊性错构瘤。

A case of genital folliculosebaceous cystic hamartoma with an unique aggregated manifestation.

作者信息

Hamada Manabu, Kiryu Hiromaro, Satoh Emiko, Moroi Yoichi, Urabe Kazunori, Furue Masutaka

机构信息

Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

出版信息

J Dermatol. 2006 Mar;33(3):191-5. doi: 10.1111/j.1346-8138.2006.00044.x.

Abstract

Folliculosebaceous cystic hamartoma (FCH) is a recently recognized cutaneous hamartoma composed of follicular, sebaceous and mesenchymal components, and usually occurring on the head and neck. We describe herein a case of FCH with an unique aggregated manifestation in a genital lesion. The patient was a 40-year-old woman with a genital lesion composed of a pedunculated nodule, a dome-shaped nodule and a subcutaneous nodule, measuring 5 cm in the greatest dimension. The largest, pedunculated nodule was histologically composed of an infundibulo-cystic structure with follicular, sebaceous and mesenchymal elements accompanied by cystic structures of various sizes lined by stratified squamous epithelium and follicular germinative cells suggesting follicular cysts. The dome-shaped nodule consisted of anastomosing strands of epithelial cells with follicular components. The subcutaneous nodule had two components, an infundibulo-cystic structure and a cyst lined by squamous epithelium. In our case, the unusual clinical feature of large and multiple nodules was due to the presence of several prominent hamartomatous cystic structures with FCH. This is the third case of giant FCH. The clinical presentation and location of giant FCH is unusual.

摘要

毛囊皮脂腺囊性错构瘤(FCH)是一种最近才被认识的皮肤错构瘤,由毛囊、皮脂腺和间充质成分组成,通常发生于头颈部。我们在此描述一例FCH在生殖器病变中呈现独特聚集表现的病例。患者为一名40岁女性,生殖器病变由一个带蒂结节、一个圆顶形结节和一个皮下结节组成,最大径为5cm。最大的带蒂结节在组织学上由一个漏斗状囊性结构组成,伴有毛囊、皮脂腺和间充质成分,还有各种大小的囊性结构,内衬复层鳞状上皮和毛囊生发细胞,提示毛囊囊肿。圆顶形结节由带有毛囊成分的上皮细胞吻合索组成。皮下结节有两个成分,一个漏斗状囊性结构和一个内衬鳞状上皮的囊肿。在我们的病例中,大的和多发结节这种不寻常的临床特征是由于存在几个突出的伴有FCH的错构瘤性囊性结构。这是第三例巨大型FCH。巨大型FCH的临床表现和部位都不寻常。

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