Franciosi Ellen, Blankenship Kaitlin, Houk Laura, Rashighi Mehdi
Dermatology, University of Massachusetts Medical School, Worcester, Massachusetts, USA.
Dermatology, University of Massachusetts Medical School, Worcester, Massachusetts, USA
BMJ Case Rep. 2020 Apr 23;13(4):e234111. doi: 10.1136/bcr-2019-234111.
An 80-year-old woman presented with a several-year history of progressive hair loss and scalp pruritus. No other rashes or muscle weakness were noted on examination. Scalp biopsy showed interface dermatitis, dense perivascular and periadnexal lymphocytic infiltrate, mucin and scarring alopecia. Laboratory analysis did not show evidence of myositis. The patient was started on hydroxychloroquine for possible cutaneous lupus erythematosus. On follow-up, she presented with a new violaceous rash on the superior eyelids and a well-defined oval patch on the mid-hard palate suspicious for dermatomyositis. Myositis-specific autoantibodies revealed presence of anti-transcriptional intermediary factor-1γ (anti-TIF1γ) in the serum. Anti-TIF1γ autoantibody-positive dermatomyositis is a newly recognised subtype of dermatomyositis that is highly associated with amyopathic disease and has an increased risk of malignancy, making prompt diagnosis crucial. This case highlights the utility of a thorough oral exam in patients suspected to have connective tissue disease as the distinctive ovoid palatal patch is nearly pathognomonic for anti-TIF1γ dermatomyositis.
一名80岁女性，有多年进行性脱发和头皮瘙痒病史。检查未发现其他皮疹或肌肉无力。头皮活检显示界面性皮炎、密集的血管周围和腺周淋巴细胞浸润、粘蛋白和瘢痕性脱发。实验室分析未显示肌炎证据。该患者开始服用羟氯喹，怀疑可能患有皮肤型红斑狼疮。随访时，她上眼睑出现新的紫红色皮疹，硬腭中部有一个边界清晰的椭圆形斑块，怀疑为皮肌炎。肌炎特异性自身抗体检测显示血清中存在抗转录中介因子1γ（抗TIF1γ）。抗TIF1γ自身抗体阳性的皮肌炎是一种新认识的皮肌炎亚型，与无肌病性疾病高度相关，且恶性肿瘤风险增加，因此及时诊断至关重要。该病例强调了对疑似结缔组织病患者进行全面口腔检查的作用，因为这种独特的卵圆形腭部斑块几乎是抗TIF1γ皮肌炎的特征性表现。
