Department of Pathology, Guizhou Provincial People's Hospital, Guiyang, Guizhou Province, China.
BMC Nephrol. 2024 Sep 18;25(1):310. doi: 10.1186/s12882-024-03748-y.
Behcet's disease (BD) is an inflammatory disorder of unknown cause that is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Local vasculitis can cause damage to the visceral system, but it is rare in kidney patients, especially those with IgA nephropathy (IgAN). In China, a small number of related cases have been reported. Here we present a case of co-occurrence of BD and IgAN.
An 18-year-old female who presented with a history of recurrent oral ulcers was found ten years ago. Four years later, the patient presented with reddish nodules on the skin of both lower limbs and then presented with vulvar ulcers. This patient was clinically diagnosed with Behcet's disease after left calf skin biopsy and presented severe proteinuria and hematuria during this period. IgAN was diagnosed after percutaneous renal biopsy. The patient was treated with hormonal, anti-inflammatory, immunomodulatory, kidney protective, and protein-lowering urine agents. After 3 years of follow-up, the patient reappears oral ulcers, reddish nodules on the skin of both lower limbs and renal dysfunction.
BD is less common in China and is clinically prone to missed diagnosis and misdiagnosis. BD with IgAN is rarer. We should regularly pay attention to the routine urine and renal function of BD patients for early detection and treatment and to prevent further progression of the disease.
贝赫切特病（BD）是一种病因不明的炎症性疾病，其特征为反复发作的口腔溃疡、生殖器溃疡、葡萄膜炎和皮肤损伤。局部血管炎可导致内脏系统损伤，但在肾脏患者中很少见，尤其是在 IgA 肾病（IgAN）患者中。在中国，有少数相关病例报道。这里我们报告一例 BD 和 IgAN 同时发生的病例。
一名 18 岁女性，十年前出现反复发作的口腔溃疡。四年后，患者下肢皮肤出现红色结节，随后出现外阴溃疡。该患者经左小腿皮肤活检后临床诊断为贝赫切特病，期间出现严重蛋白尿和血尿。经皮肾活检诊断为 IgAN。患者接受了激素、抗炎、免疫调节、肾脏保护和降蛋白尿治疗。随访 3 年后，患者再次出现口腔溃疡、下肢皮肤红色结节和肾功能不全。
BD 在我国较少见，临床易误诊和漏诊。BD 合并 IgAN 更为罕见。我们应定期关注 BD 患者的常规尿液和肾功能，以便早期发现和治疗，防止疾病进一步进展。
