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[多发性漏斗瘤]

[Multiple infundibuloma].

作者信息

Cribier B, Waskievicz W, Heid E

机构信息

Clinique Dermatologique, Faculté de Médecine, Université Louis-Pasteur, Strasbourg, France.

出版信息

Ann Dermatol Venereol. 1991;118(4):281-5.

PMID:2069339
Abstract

A 42-year-old man presented with multiple hypopigmented macules, 4 to 10 mm wide, irregularly shaped and symmetrically distributed on the neck and face, with a 10 years duration. Histological examinations showed a plate-like epithelial proliferation below the epidermis. The cells had a pale cytoplasm, with few PAS positive granules. In the lower part of the proliferation the peripheral cells were palisading and surrounded by a dense elastic network. Serial sections showed connections with the normal outer root sheath of the neighbour follicles, and with the epidermis. This pattern is very characteristic of Mehregan's tumor of follicular infundibulum. Four distinctive presentations of this tumor can be observed, in spite of its rarity: multiple macules of the upper chest or face (4 cases have previously been described), tumors of follicular infundibulum occurring in Cowden's disease or in the natural course of organoid nevi, and the most common solitary form of the face. With their striking clinical features, the eruptive or multiple forms of this tumor should be recognized as a distinctive clinical entity. Possible transformation into basal cell carcinoma has been described in one of these cases, and long time supervision should be recommended.

摘要

一名42岁男性,颈部和面部出现多个色素减退斑,直径4至10毫米,形状不规则,对称分布,病程10年。组织学检查显示表皮下有板状上皮增生。细胞胞质淡染,PAS阳性颗粒较少。在增生的下部,周边细胞呈栅栏状排列,并被致密的弹性网络包围。连续切片显示与相邻毛囊的正常外根鞘以及表皮相连。这种模式是毛囊漏斗部梅雷甘肿瘤的非常典型特征。尽管该肿瘤罕见,但可观察到四种不同表现:上胸部或面部的多发性斑疹(此前已描述4例)、发生于考登病或类器官痣自然病程中的毛囊漏斗部肿瘤,以及最常见的面部孤立形式。鉴于其显著的临床特征,该肿瘤的发疹性或多发性形式应被视为一种独特的临床实体。其中1例已描述可能转化为基底细胞癌,因此建议长期随访。

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