指甲淀粉样变性瘤:两例新病例报告

IF 1.4 Q3 DERMATOLOGY Skin Appendage Disorders Pub Date : 2023-06-01 Epub Date: 2023-02-01 DOI:10.1159/000529091
Frederico Bonito, Athanassios Kolivras, Ursula Sass, Bertrand Richert
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引用次数: 0

摘要

简介淀粉样变性是一组以异常不溶性蛋白质在不同组织的细胞外沉积为特征的疾病。淀粉样变性瘤是一种没有全身性淀粉样变性的淀粉样蛋白局部肿瘤沉积,在不同的解剖部位均有描述。我们报告了两例指甲部位的淀粉样瘤病例,并对这一最新描述的实体病例进行了深入研究:两个病例均表现为无症状、缓慢生长的结节,位于脚趾远端甲床下方,伴有甲沟炎。组织病理学特征是,两名患者的真皮层和皮下组织中均有刚果红阳性、均质、无定形和嗜酸性物质沉积,并伴有浆细胞聚集。在这两个病例中,广泛的检查排除了全身性淀粉样变性。治疗以局部切除为主,随访一年未发现局部复发或发展为全身性淀粉样变性:结论:这是首次关于指甲淀粉样变性的报道。结论:这是首次报道指甲淀粉样变性,其临床和组织病理学表现与皮肤淀粉样变性相似。局部切除似乎是一种有效的治疗方式,但仍需长期随访,以排除复发、伴发边缘B细胞淋巴瘤或发展为全身性淀粉样L淀粉样变性。
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Nail Amyloidoma: Two Case Reports of a New Entity.

Introduction: Amyloidosis is a group of diseases characterized by extracellular deposits of abnormal insoluble proteins in different tissues. Amyloidoma is a localized tumoral deposit of amyloid in the absence of systemic amyloidosis, and it has been described in different anatomic sites. We report two cases of amyloidoma in the nail unit and provide insights into this recently described entity.

Case presentation: Both cases presented as an asymptomatic, slowly growing nodule underneath the distal nail bed of a toe with associated onycholysis. Histopathology was characterized in both patients by the presence of deposits of Congo red-positive, homogeneous, amorphous, and eosinophilic material within the dermis and subcutaneous tissue admixed with aggregates of plasma cells. In both cases, an extensive workup excluded systemic amyloidosis. Treatment was based on local excision, and no local recurrence or progression to systemic amyloidosis was observed at 1 year of follow-up.

Conclusion: These are the first reports of amyloidomas of the nail unit. The clinical and histopathological presentations parallel those of an amyloidoma affecting the skin. Local excision seems to be an efficient treatment modality, but long-term follow-up is warranted in order to exclude recurrence, an associated marginal B-cell lymphoma, or progression to systemic amyloid L amyloidosis.

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CiteScore
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自引率
10.00%
发文量
69
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