Aung Thanda, Celestin Mia
Rheumatology, University of California Los Angeles, David Geffen School of Medicine, Los Angeles, USA.
Cureus. 2025 Apr 17;17(4):e82439. doi: 10.7759/cureus.82439. eCollection 2025 Apr.
Sjögren's syndrome is an autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, primarily affecting the lacrimal and salivary glands, resulting in dry eyes and mouth. We report a case of a 22-year-old female with Sjögren's syndrome (diagnosed at age 14), who developed chilblain-like lesions with histopathological features of tumid lupus. The patient presented with low-grade fever and painful erythematous papules on the fingers, toes, ears, face, and trunk. Serological workup revealed multiple autoantibodies (anti-SSA (Sjögren's-syndrome-related antigen A), anti-SSB (Sjögren's-syndrome-related antigen B), anti-centromere, anti-U1RNP (anti-U1 ribonucleoprotein), ANA (antinuclear antibody), and rheumatoid factor). Skin biopsy demonstrated interface dermatitis with lymphoplasmacytic infiltration and increased dermal mucin. After an inadequate response to conventional therapy (hydroxychloroquine, methotrexate, prednisone, and topical treatments), the patient showed significant improvement with belimumab. This case highlights the overlap between Sjögren's syndrome and lupus spectrum disorders. It demonstrates the potential efficacy of B-cell-targeted therapy in managing refractory cutaneous manifestations of autoimmune overlap syndromes.
干燥综合征是一种自身免疫性疾病，其特征为外分泌腺的淋巴细胞浸润，主要累及泪腺和唾液腺，导致眼干和口干。我们报告一例22岁女性干燥综合征患者（14岁时确诊），该患者出现了具有肿胀性狼疮组织病理学特征的冻疮样皮损。患者表现为低热，手指、脚趾、耳部、面部和躯干出现疼痛性红斑丘疹。血清学检查发现多种自身抗体（抗SSA（干燥综合征相关抗原A）、抗SSB（干燥综合征相关抗原B）、抗着丝点抗体、抗U1RNP（抗U1核糖核蛋白）、抗核抗体和类风湿因子）。皮肤活检显示界面性皮炎伴淋巴细胞浆细胞浸润及真皮黏蛋白增加。在对传统治疗（羟氯喹、甲氨蝶呤、泼尼松和局部治疗）反应不佳后，患者使用贝利尤单抗后有显著改善。该病例突出了干燥综合征与狼疮谱系疾病之间的重叠。它证明了B细胞靶向治疗在管理自身免疫重叠综合征难治性皮肤表现方面的潜在疗效。
