Primary skin amyloidosis is a chronic skin disease in which amyloid deposits in the normal skin tissues without involving other organs. At present, the causes and mechanisms of morbidity have not been fully elucidated. There are few clinical reports of nodular skin amyloidosis, and the domestic reports are mostly limited cases. This study reported a rare case of a 46-year-old woman with primary localized cutaneous nodular amyloidosis (PLCNA). The patient presented with features of lymphatic malformation, a plexiform nodule of small blisters. Histological examination revealed amyloid deposits involving the superficial and deep dermis with a small number of plasma cells. Further examinations did not reveal evidence of systemic involvement, indicating a PLCNA. The presentation as lymphatic malformation lesions illustrates the importance of clinical pathology. Nodular amyloidosis typically manifests as single or multiple yellow-brown nodules or plaques of a few millimeters to several centimeters. The center of the nodule sometimes shows atrophy and relaxation or forms a bullous. It is recommended to perform a pathological examination to confirm the diagnosis to distinguish it from lymphatic malformation.

中文翻译：

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表现为淋巴管畸形的原发性局限性皮肤结节性淀粉样变：一例报告

原发性皮肤淀粉样变性是一种淀粉样蛋白沉积在正常皮肤组织而不累及其他器官的慢性皮肤病。目前，发病原因和机制尚未完全阐明。结节性皮肤淀粉样变性的临床报道较少，国内报道多为有限病例。这项研究报道了一名 46 岁女性的罕见病例，她患有原发性局限性皮肤结节性淀粉样变性 (PLCNA)。患者表现出淋巴管畸形、丛状小水疱结节的特征。组织学检查发现淀粉样蛋白沉积物涉及真皮浅层和深层，并有少量浆细胞。进一步检查没有发现全身受累的证据，表明有 PLCNA。淋巴管畸形病变的表现说明了临床病理学的重要性。结节性淀粉样变性通常表现为单个或多个几毫米至几厘米的黄棕色结节或斑块。结节中心有时呈萎缩松弛或形成大疱。建议进行病理检查明确诊断，以与淋巴管畸形相鉴别。