Indu Sudip, Roy Indranil Deb, Tewari Rohit, Pramanik Suman
Division of Oral Pathology, Army Dental Centre (R and R), New Delhi, India.
Armed Forces Dental Clinic, New Delhi, India.
J Oral Maxillofac Pathol. 2021 Sep-Dec;25(3):559. doi: 10.4103/jomfp.JOMFP_227_20. Epub 2022 Jan 11.
Amyloidosis is a distressing and infrequent condition caused by accumulation of abnormally folded proteins as aggregates in the extracellular tissue spaces of the body, leading to destruction of organ structure and function. Presentation of Oral Amyloidosis is generally a rare phenomenon, tongue being the most common site to manifest the disease process. Accurate recognition of the amyloid protein and its sub typing is absolutely critical for clinical management and to assess prognosis such as to avoid misdiagnosis and unwanted, potentially harmful treatment.
We report a case of 84 year old male patient with an enlarged tongue, who presented with burning sensation and a facial violaceous papules with no other known systemic findings. Incisional biopsy revealed amorphous deposits confirmed for amyloid by congo red stain and Immunohistochemistry.
The suspicion of Amyloidosis should always be kept in mind whenever a solitary enlargement of the tongue is noticed. Close follow-up and an entire panel of investigations is mandatory, both to manage recurrences and to monitor the possible advancement of the disease into a full blown systemic form.
淀粉样变性是一种令人痛苦且罕见的病症,由异常折叠的蛋白质聚积在身体细胞外组织间隙中形成聚集体所致,会导致器官结构和功能破坏。口腔淀粉样变性的表现通常较为罕见,舌头是该疾病进程最常出现症状的部位。准确识别淀粉样蛋白及其亚型对于临床管理和评估预后绝对至关重要,例如可避免误诊以及不必要的、可能有害的治疗。
我们报告一例84岁男性患者,其舌头肿大,伴有烧灼感和面部紫红色丘疹,无其他已知的全身症状。切开活检显示刚果红染色和免疫组织化学证实为淀粉样蛋白的无定形沉积物。
每当发现舌头单独肿大时,应始终怀疑淀粉样变性。密切随访和进行全面的检查是必要的,这既能处理复发情况,又能监测疾病可能发展为全面的全身性形式。