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小汗腺血管瘤样错构瘤:18例临床病理分析

Eccrine Angiomatous Hamartoma: A Clinicopathologic Review of 18 Cases.

作者信息

Patterson Andrew T, Kumar Monique G, Bayliss Susan J, Witman Patricia M, Dehner Louis P, Gru Alejandro A

机构信息

*Ohio State University College of Medicine, Columbus, OH; †Department of Dermatology, Emory University School of Medicine, Atlanta, GA; ‡Division of Dermatology, Department of Internal Medicine, Washington University School of Medicine, St. Louis, MO; §Division of Dermatology, Nationwide Children's Hospital, Columbus, OH; ¶Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO; and ‖Department of Pathology, Ohio State University Wexner Medical Center, Columbus, OH.

出版信息

Am J Dermatopathol. 2016 Jun;38(6):413-7. doi: 10.1097/DAD.0000000000000430.

DOI:10.1097/DAD.0000000000000430
PMID:26760684
Abstract

BACKGROUND

Eccrine angiomatous hamartoma (EAH) is a benign cutaneous lesion defined by the proliferation of hamartomatous eccrine and capillary-like vascular elements in the dermis. However, the epidemiologic, morphologic, and histopathologic aspects of this uncommon disorder have yet to be fully delineated.

METHODS

The authors retrospectively reviewed 18 EAH cases (including 14 accompanying skin biopsy specimens) diagnosed at 4 American university hospitals from 1996 to 2014.

RESULTS

Patients ranged from 3 days to 84 years at time of diagnosis with a median age of 15 years. A male:female ratio of 11:7 was observed. Sixty-seven percent of cases presented in the extremities, but lesions in the trunk and head/neck regions also occurred. Four patients had multiple lesions, and 2 displayed a segmental pattern. Histologically, dermal vascular dilatation and acanthosis often accompanied EAH's typical eccrine and vascular comingling. One individual developed EAH at the site of a recurrent squamous cell carcinoma after previous excision.

CONCLUSIONS

Although previously thought to occur primarily as a solitary angiomatous-appearing malformation on the extremities of children, EAH may develop with some frequency in adults and may manifest in a multifocal linear distribution. The authors also raise additional histopathologic consideration in support of the vascular theory of histogenesis for this condition.

摘要

背景

小汗腺血管瘤性错构瘤(EAH)是一种良性皮肤病变,其定义为真皮内错构瘤性小汗腺和毛细血管样血管成分的增生。然而,这种罕见疾病的流行病学、形态学和组织病理学方面尚未完全阐明。

方法

作者回顾性分析了1996年至2014年在美国4所大学医院诊断的18例EAH病例(包括14例伴有皮肤活检标本)。

结果

患者诊断时年龄从3天至84岁不等,中位年龄为15岁。观察到男女比例为11:7。67%的病例出现在四肢,但躯干和头颈部也有病变。4例患者有多处病变,2例表现为节段性分布。组织学上,真皮血管扩张和棘皮症常伴随EAH典型的小汗腺和血管混合。1例患者在先前切除复发性鳞状细胞癌的部位发生了EAH。

结论

尽管此前认为EAH主要表现为儿童四肢孤立的血管瘤样畸形,但它在成人中也可能频繁发生,并且可能表现为多灶性线性分布。作者还提出了额外的组织病理学考虑因素,以支持这种疾病的血管发生理论。

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