■淀粉样变是一组疾病,其特征是在不同组织中异常不溶性蛋白质的细胞外沉积。淀粉样瘤是在没有系统性淀粉样变性的情况下淀粉样蛋白的局部肿瘤沉积,它已经在不同的解剖部位被描述过。我们报告了指甲单元中的两例淀粉样瘤,并提供了对这一最近描述的实体的见解。
■这两种情况都表现为无症状,在脚趾远端甲床下缓慢生长的结节,并伴有甲溶解。两名患者的组织病理学特征是存在刚果红阳性沉积物,同质,无定形,真皮和皮下组织内的嗜酸性物质与浆细胞聚集体混合。在这两种情况下,广泛的检查排除了系统性淀粉样变性。治疗是基于局部切除,随访1年,未观察到局部复发或进展为系统性淀粉样变性.
■这些是指甲单位的淀粉样瘤的首次报道。临床和组织病理学表现与影响皮肤的淀粉样瘤相似。局部切除似乎是一种有效的治疗方式,但长期随访是必要的,以排除复发,相关的边缘B细胞淋巴瘤,或进展为系统性淀粉样蛋白L淀粉样变性。
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.