Fastner Suzanne L, Clarke Jennie T
Dermatology, Medical College of Wisconsin, Milwaukee, USA.
Dermatology, University of Utah School of Medicine, Salt Lake City, USA.
Cureus. 2024 Jul 15;16(7):e64609. doi: 10.7759/cureus.64609. eCollection 2024 Jul.
Good syndrome (GS) is a rare condition characterized by thymoma and immune deficiency with a poorly understood mechanism in which patients have reduced immunoglobulin levels and circulating B-cells along with impaired T-cell function. GS is often accompanied by autoimmune and inflammatory conditions, and in this report, we present a case of refractory oral lichen planus (OLP) preceding the diagnosis of GS. In this case, a patient with a history of OLP was diagnosed with GS and common variable immunodeficiency (CVID) following thymectomy and was treated with intravenous immunoglobin (IVIG). Additionally, he was found to have pure red cell aplasia managed with cyclosporine. His oral symptoms worsened, and he presented to dermatology. Treatment was initiated with topical clobetasol and tacrolimus for his OLP, and fluconazole was started for concomitant oral candidiasis. His OLP has remained under satisfactory control with this regimen; however, he requires close surveillance for malignancy given his increased risk of oral squamous cell carcinoma (OSCC) with immunosuppression and active OLP. Although rare, clinicians should be aware of GS and its association with erosive OLP along with the heightened risk of infection in these patients.
古德综合征(GS)是一种罕见疾病,其特征为胸腺瘤和免疫缺陷,发病机制尚不清楚,患者免疫球蛋白水平降低、循环B细胞减少,同时T细胞功能受损。GS常伴有自身免疫和炎症性疾病,在本报告中,我们介绍一例在GS诊断之前出现的难治性口腔扁平苔藓(OLP)病例。在此病例中,一名有OLP病史的患者在胸腺切除术后被诊断为GS和常见变异型免疫缺陷(CVID),并接受了静脉注射免疫球蛋白(IVIG)治疗。此外,发现他患有纯红细胞再生障碍性贫血,采用环孢素进行治疗。他的口腔症状加重,于是到皮肤科就诊。针对其OLP开始使用外用氯倍他索和他克莫司进行治疗,并开始使用氟康唑治疗伴发的口腔念珠菌病。采用该治疗方案后,他的OLP一直得到满意控制;然而,鉴于他因免疫抑制和活动性OLP而患口腔鳞状细胞癌(OSCC)的风险增加,需要密切监测是否发生恶性肿瘤。尽管罕见,但临床医生应了解GS及其与糜烂性OLP的关联,以及这些患者感染风险的增加。