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多发性毛发上皮瘤综合征:一例报告

Multiple Trichoepithelioma Syndrome: A Case Report.

作者信息

Guerreiro Frederico P, Martins Ana I, Costa E Silva Joana, Teixeira Nelson, Ramos José N

机构信息

Plastic, Reconstructive, and Maxillofacial Surgery, Centro Hospitalar de Lisboa Ocidental, Lisbon, PRT.

出版信息

Cureus. 2023 Aug 4;15(8):e42930. doi: 10.7759/cureus.42930. eCollection 2023 Aug.

DOI:10.7759/cureus.42930
PMID:37667697
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10475157/
Abstract

Multiple trichoepithelioma syndrome is a rare entity, and little is known about its epidemiological features. Patients usually present with multiple nonsuspicious skin lesions. Surgical excision is the mainstay of treatment, and diagnosis is usually made after the first pathology report. Once the diagnosis is established, patients are kept under clinical surveillance, and surgery is performed again if tumor burden and/or size justifies it. The authors present a male patient who presented to our outpatient clinic for the first time in 36 years without any relevant medical history, medication, or allergies. The patient had complaints of multiple skin lesions spreading across the head and neck regions. Surgical excision of the affected area and resurfacing using local advancement flaps were performed. Pathology reports were always consistent with trichoepitheliomas. No pathology of spiradenoma or cylindroma was ever reported. Usually, tumors are small enough for simple excision and primary closure. However, in the presented case, the size of the tumor and the involvement of central facial aesthetic units demanded a more complex approach.

摘要

多发性毛发上皮瘤综合征是一种罕见的病症,对其流行病学特征了解甚少。患者通常表现为多发性无可疑的皮肤病变。手术切除是主要的治疗方法,诊断通常在首次病理报告后做出。一旦确诊,患者需接受临床监测,如果肿瘤负荷和/或大小表明有必要,则再次进行手术。作者介绍了一名男性患者,该患者36年来首次到我们的门诊就诊,无任何相关病史、用药史或过敏史。患者主诉头部和颈部有多发性皮肤病变。对受累区域进行了手术切除,并使用局部推进皮瓣进行了创面修复。病理报告始终与毛发上皮瘤一致。从未报告过汗管瘤或圆柱瘤的病理情况。通常,肿瘤较小,可进行简单切除和一期缝合。然而,在本病例中,肿瘤的大小以及面部中央美学单元的受累情况需要采用更复杂的方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/26ae/10475157/d35191cb9c03/cureus-0015-00000042930-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/26ae/10475157/d35191cb9c03/cureus-0015-00000042930-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/26ae/10475157/d35191cb9c03/cureus-0015-00000042930-i01.jpg

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Multiple Trichoepithelioma Syndrome: A Case Report.多发性毛发上皮瘤综合征:一例报告
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本文引用的文献

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Brooke-Spiegler Syndrome and Phenotypic Variants: An Update.布鲁克-施皮格勒综合征及其表型变异:最新进展
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