单侧孤立性外耳道原发性淀粉样变。
Unilateral Isolated Primary Cutaneous Amyloidosis of the External Auditory Canal.
机构信息
Department of Sense Organs, Sapienza University of Rome, Rome, Italy.
Department of Molecular Medicine, Sapienza University of Rome, Italy.
出版信息
J Int Adv Otol. 2020 Dec;16(3):467-469. doi: 10.5152/iao.2020.7570.
Isolated primary cutaneous amyloidosis (PCA) of the external ear is extremely rare. We describe the case of a 65-year-old woman presenting with itching within the left external auditory canal (EAC). Otoscopy revealed a 3 mm whitish lesion involving the cartilaginous portion of the left EAC. The lesion was excised. Histological and immunohistochemical features were consistent with keratinic amyloidosis. A clinical workup was negative for systemic amyloidosis. As far as we know, only nine cases of PCA exclusively involving the EAC have been reported. The frequent occurrence of itching in these patients and the keratinic nature of the amyloid support the role of chronic stimulation/irritation in the pathogenesis of isolated amyloidosis the EAC.
原发性皮肤淀粉样变性（PCA）孤立于耳部者非常罕见。我们报告了 1 例 65 岁女性，因左耳外耳道（EAC）内瘙痒就诊。耳镜检查发现 1 个 3 毫米大小的乳白色病变累及左侧 EAC 的软骨部分。病变被切除。组织学和免疫组化特征与角蛋白淀粉样变性一致。全身性淀粉样变性的临床检查为阴性。据我们所知，仅有 9 例 PCA 仅累及 EAC 的病例报告。这些患者常出现瘙痒，且淀粉样物质具有角蛋白性质，这支持慢性刺激/刺激在 EAC 孤立性淀粉样变性发病机制中的作用。
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