Hile Grace A, Werth Victoria P
University of Michigan, Ann Arbor.
Corporal Michael J. Crescenz Department of Veterans Affairs Medical Center and the University of Pennsylvania, Philadelphia.
Arthritis Rheumatol. 2025 Jan;77(1):1-11. doi: 10.1002/art.42983. Epub 2024 Oct 29.
A 29-year-old female presented to a rheumatology-dermatology clinic with a pruritic rash that began 6 months prior, after a viral illness. She had previously been diagnosed with eczema and treated with antihistamines and topical steroids without improvement. She also noted fatigue, hair loss, and severe scalp pruritus. Physical examination was notable for violaceous periorbital edema, scaly erythematous papules on the metacarpophalangeal joints of bilateral hands, dilated capillaries of the proximal nail folds, scaly plaques on bilateral elbows, and excoriated erythematous plaques on upper chest, back and hips. The patient reported no muscle weakness, and strength testing and creatinine phosphokinase were normal. Magnetic resonance imaging of the thigh showed no evidence of inflammation or edema. Antibody testing was negative. A diagnosis of clinically amyopathic dermatomyositis was made. Computed tomography scans of the chest, abdomen and pelvis, colonoscopy, and mammogram showed no evidence of cancer. The patient was initiated on methotrexate. Her cutaneous manifestations persisted with debilitating intractable pruritus, and thus, she was transitioned to mycophenolate mofetil, again with minimal improvement. Intravenous immunoglobulin was not approved by insurance given the lack of muscle involvement in her disease. This patient's case highlights a common clinical scenario in rheumatology and dermatology and raises several important issues related to the immunologic underpinnings of cutaneous lupus erythematosus (CLE) and dermatomyositis (DM): What is the role of type I interferon (IFN) in triggering skin disease in CLE and DM? What is the role of IFN in the pathogenesis of skin inflammation in CLE and DM? Can we apply what we know about IFN-targeted therapeutics in CLE and DM to develop better treatments for skin disease?
一名29岁女性因瘙痒性皮疹就诊于风湿科-皮肤科门诊，该皮疹始于6个月前一次病毒感染后。她此前被诊断为湿疹，曾接受抗组胺药和外用类固醇治疗，但无改善。她还伴有疲劳、脱发和严重的头皮瘙痒。体格检查发现有紫罗兰色眶周水肿、双手掌指关节处鳞屑性红斑丘疹、近端甲襞毛细血管扩张、双侧肘部鳞屑性斑块以及上胸部、背部和臀部有抓痕的红斑性斑块。患者自述无肌肉无力，肌力测试和肌酸磷酸激酶均正常。大腿磁共振成像未显示炎症或水肿迹象。抗体检测为阴性。诊断为临床无肌病性皮肌炎。胸部、腹部和骨盆的计算机断层扫描、结肠镜检查和乳房X线摄影均未发现癌症迹象。患者开始使用甲氨蝶呤治疗。她的皮肤表现持续存在，伴有使人衰弱的顽固性瘙痒，因此，她改用霉酚酸酯治疗，改善仍甚微。鉴于她的疾病无肌肉受累，静脉注射免疫球蛋白未获保险批准。该患者的病例突出了风湿科和皮肤科常见的临床情况，并引发了几个与皮肤红斑狼疮（CLE）和皮肌炎（DM）的免疫基础相关的重要问题：I型干扰素（IFN）在CLE和DM引发皮肤疾病中起什么作用？IFN在CLE和DM皮肤炎症的发病机制中起什么作用？我们能否将我们对CLE和DM中IFN靶向治疗的了解应用于开发更好的皮肤病治疗方法？
