Alahmadi Ghadeer G, El-Desoky Sherif M, Al Zahrani Reem A, Kari Jameela A
Pediatrics, King Abdulaziz University Faculty of Medicine, Jeddah, SAU.
Pediatrics Nephrology, King Abdulaziz University Faculty of Medicine, Jeddah, SAU.
Cureus. 2023 Jan 29;15(1):e34332. doi: 10.7759/cureus.34332. eCollection 2023 Jan.
We report the case of an adolescent girl with frequent hospital admissions for severe eczematous skin rashes with recurrent epistaxis and chest infections. Investigations revealed persistent severely elevated serum total immunoglobulin E (IgE) levels but normal levels of other immunoglobulins, suggesting hyper-IgE syndrome. The first skin biopsy revealed superficial dermatophytic dermatitis (tinea corpora). Another biopsy performed after six months revealed a prominent basement membrane with dermal mucin, suggesting an underlying autoimmune disease. Her condition was complicated by proteinuria, hematuria, hypertension, and edema. A kidney biopsy revealed class IV lupus nephritis, according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS). Based on the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria, she was diagnosed with systemic lupus erythematosus (SLE). She was first administered with intravenous pulse methylprednisolone (600 mg/m) for three consecutive days, followed by oral prednisolone (40 mg/m) daily, mycophenolate mofetil tablets (600 mg/m/dose) twice daily, hydroxychloroquine (200 mg) once daily, and three classes of antihypertensive medications. She maintained normal renal functions with no lupus morbidity for 24 months, then rapidly progressed to end-stage kidney disease, and was then started on three to four sessions of regular hemodialysis per week. Hyper-IgE is known to be a marker of immune dysregulation as it facilitates the generation of immune complexes (ICs) that mediate lupus nephritis and juvenile SLE. Regardless of the different factors that are impacting the production of IgE, the present case illustrated that juvenile patients with SLE may have increased IgE levels, indicating that higher IgE levels might have a role in lupus pathogenesis and prognosis. The mechanisms regarding the increased levels of IgE in subjects with lupus need further investigation. Further studies are thus required to assess the incidence, prognosis, and possible new specific management for hyper-IgE in juvenile SLE.
我们报告了一名青春期女孩的病例，她因严重的湿疹性皮疹、反复鼻出血和胸部感染而频繁住院。检查发现血清总免疫球蛋白E（IgE）水平持续严重升高，但其他免疫球蛋白水平正常，提示高IgE综合征。首次皮肤活检显示浅表皮肤癣菌性皮炎（体癣）。六个月后进行的另一次活检显示基底膜突出伴真皮粘蛋白，提示潜在的自身免疫性疾病。她的病情因蛋白尿、血尿、高血压和水肿而复杂化。根据国际肾脏病学会/肾脏病理学会（ISN/RPS）的标准，肾脏活检显示为IV级狼疮性肾炎。根据美国风湿病学会/欧洲抗风湿病联盟（ACR/EULAR）标准，她被诊断为系统性红斑狼疮（SLE）。她首先连续三天静脉注射脉冲甲基强的松龙（600 mg/m），随后每天口服强的松龙（40 mg/m）、霉酚酸酯片（600 mg/m/剂量）每日两次、羟氯喹（200 mg）每日一次，以及三类降压药物。她维持正常肾功能24个月无狼疮发病，然后迅速进展为终末期肾病，随后开始每周进行三到四次定期血液透析。高IgE已知是免疫失调的标志物，因为它促进介导狼疮性肾炎和青少年SLE的免疫复合物（IC）的产生。无论影响IgE产生的不同因素如何，本病例表明SLE青少年患者可能有IgE水平升高，表明较高的IgE水平可能在狼疮发病机制和预后中起作用。狼疮患者中IgE水平升高的机制需要进一步研究。因此，需要进一步研究来评估青少年SLE中高IgE的发病率、预后和可能的新的特异性治疗方法。
