Gomes Sara, Santos Sara, Silva Monica, Ferreira Joana, Ponte Teresinha
Internal Medicine, Centro Hospitalar Barreiro-Montijo, Barreiro, PRT.
Cureus. 2023 Jan 7;15(1):e33480. doi: 10.7759/cureus.33480. eCollection 2023 Jan.
Amyloidosis is a mainly systemic rare disease and its localized presentation is even less frequent. Systemic forms are often associated with other conditions or entities (such as neoplasms), and a correct etiological study of patients with this disease is essential. A localized presentation has a better prognosis compared to systemic forms, which underlines the importance of a correct diagnosis. Primary localized amyloidosis in the head and neck region is a rare entity. Primary amyloidosis localized to the sinonasal tract is extremely rare. Although uncommon, case reports have been increasing in the face of new endoscopic and imaging techniques, and the report of these cases is crucial for a better characterization of this entity. Symptoms may include epistaxis, nasal obstruction, facial deformity, and vision changes. A 72-year-old female with a history of frequent nasal congestion with two years' duration showed no improvement following symptom management. She was referred for nasal endoscopy and biopsy, thereby confirming the diagnosis of amyloidosis. Further diagnostic testing did not show evidence of systemic disease. This case report was previously presented as a poster presentation at the 19th European Congress of Internal Medicine in March 2021.
淀粉样变性是一种主要累及全身的罕见疾病，其局限性表现更为少见。系统性淀粉样变性常与其他疾病或病变（如肿瘤）相关，因此对该病患者进行正确的病因学研究至关重要。与系统性淀粉样变性相比，局限性淀粉样变性的预后较好，这凸显了正确诊断的重要性。头颈部原发性局限性淀粉样变性是一种罕见的病变。局限于鼻窦的原发性淀粉样变性极为罕见。尽管不常见，但随着新的内镜和影像学技术的出现，病例报告不断增加，这些病例的报告对于更好地认识这一病变至关重要。症状可能包括鼻出血、鼻塞、面部畸形和视力改变。一名72岁女性，有两年频繁鼻充血病史，症状处理后无改善。她被转诊进行鼻内镜检查和活检，从而确诊为淀粉样变性。进一步的诊断检查未发现系统性疾病的证据。该病例报告曾于2021年3月在第19届欧洲内科医学大会上以海报形式展示。
