Pacinioma of Lumbosacral Skin in Closed Spinal Dysraphism.

IF 1.6 4区 医学 Q3 DERMATOLOGY
Bryan Johnston, Katelynn Campbell
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Abstract

Closed spinal dysraphism (CSD) is a congenital condition caused by a failure in secondary neurulation during embryogenesis. CSD is associated with characteristic cutaneous stigmata often identified clinically. Rarely, such stigmata have been reported to occur with complex congenital intraspinal lipomas containing Pacinian corpuscle hyperplasia. Herein, we present our case of an asymptomatic 8-month-old female who presented with a midline lumbosacral acrochordon-like lesion. MRI revealed a midline lipomatous lesion at S2-S3 with a fibrovascular stalk likely continuous with the acrochordon. Given the absence of neurological symptoms concerning tethered cord syndrome (TCS), only the skin lesion was removed for cosmesis. Subsequent histopathological examination revealed numerous Pacinian corpuscles in a background of dermal collagen and subcutaneous adipose tissue. No features suggested a co-existent peripheral nerve sheath lesion (such as a neurofibroma). Given the spinal cord abnormalities, and the histologic features of a pure proliferation of Pacinian corpuscles, a diagnosis of the so-called "Pacinioma" was made. To the best of our knowledge, only four other cases of complex intraspinal lipomatous lesions containing Pacinioma in the setting of CSD with a tethered cord have been previously reported. This case highlights the complexity of the cutaneous stigmata of CSD.

闭合性脊椎发育不良的腰骶部皮肤acinioma。
闭合性脊柱发育不良(CSD)是一种先天性疾病,由胚胎发育过程中的继发性神经发育不全引起。CSD 与特征性的皮肤瘢痕有关,通常可在临床上识别。罕见的是,此类皮肤瘢痕还与复杂的先天性椎管内脂肪瘤(包含帕氏体增生)同时出现。在此,我们介绍了一例无症状的 8 个月大女性病例,她出现了腰骶部中线棘突样病变。核磁共振成像显示,S2-S3 中线脂肪瘤病变,其纤维血管蒂可能与棘突连续。鉴于患者没有出现有关系索综合征(TCS)的神经症状,为美观起见,只切除了皮损。随后的组织病理学检查显示,在真皮胶原和皮下脂肪组织的背景下有许多帕西尼氏体。没有任何特征表明同时存在周围神经鞘病变(如神经纤维瘤)。鉴于脊髓异常和帕西尼氏体纯增生的组织学特征,诊断结果为所谓的 "帕西尼瘤"。据我们所知,此前仅有四例脊髓内脂肪瘤病变的复杂病例,其中包括在脊髓系带断裂(CSD)的情况下出现的帕西尼瘤。本病例凸显了 CSD 皮肤症状的复杂性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
3.20
自引率
5.90%
发文量
174
审稿时长
3-8 weeks
期刊介绍: Journal of Cutaneous Pathology publishes manuscripts broadly relevant to diseases of the skin and mucosae, with the aims of advancing scientific knowledge regarding dermatopathology and enhancing the communication between clinical practitioners and research scientists. Original scientific manuscripts on diagnostic and experimental cutaneous pathology are especially desirable. Timely, pertinent review articles also will be given high priority. Manuscripts based on light, fluorescence, and electron microscopy, histochemistry, immunology, molecular biology, and genetics, as well as allied sciences, are all welcome, provided their principal focus is on cutaneous pathology. Publication time will be kept as short as possible, ensuring that articles will be quickly available to all interested in this speciality.
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