Department of Integrated Traditional Chinese and Western Medicine of Rheumatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China.
Clin Rheumatol. 2021 Sep;40(9):3687-3694. doi: 10.1007/s10067-021-05656-0. Epub 2021 Mar 12.
This study aims to analyze the clinical characteristics of juvenile primary Sjogren's syndrome (pSS) with cutaneous involvement.
We investigated the clinical and immunological characteristics of 37 children with pSS. All the patients met the 2012 American College of Rheumatology Classification Criteria for Sjogren's syndrome.
The cutaneous involvement presented in 19 children (excluding drug eruption), 16 (84.2%) female patients, and 3 (15.8%) male patients, with a mean age of 11 ± 2.68 years, 17 of whom (89.5%) had cutaneous lesions as the first symptom, with a median time of 12 months (1 day to 4 years) before the diagnosis of pSS. The cutaneous lesions included 12 cases of palpable purpura (63.2%), 5 cases of urticaria (26.3%), 2 cases of xeroderma (10.5%), 1 case of skin ulcer, 1 case of erythema nodosum, 1 case of livedo reticularis, 1 case of Raynaud's phenomenon, and 1 case of hard erythema. Children with cutaneous lesions had a higher prevalence of articular involvement (42.1% vs. 11.1%, P = 0.016), fever (47.4% vs. 5.6%, P = 0.004), ESR > 50 mm/h (47.4% vs. 11.1%, P = 0.016), and a lower prevalence of thrombocytopenia (0% vs. 27.8%, P = 0.013) and methylprednisolone pulse treatment (0% vs. 13.5%, P = 0.013), compared with pSS without cutaneous involvement.
More than half (51.3%) of the children with juvenile pSS presented with cutaneous lesions; the main cutaneous involvement was palpable purpura. Children with cutaneous lesions were more likely to have fever and arthritis, were more likely to have stronger inflammatory response, and were less likely to have serious complications. In many cases, the cutaneous lesion could be the first symptom of juvenile pSS, which could easily lead to a misdiagnosis. The possibility of pSS should be considered for children with skin lesions such as palpable purpura and urticaria, and further examinations should be carried out. Key Points • Cutaneous lesions of juvenile pSS are not uncommon and often present as the first symptom. • Palpable purpura was the most common skin lesion in juvenile pSS, followed by urticaria. • Juvenile pSS with skin lesions does not increase the risk of serious complications such as blood system damage. • Skin lesions in juvenile pSS patients may easily cause misdiagnosis.
本研究旨在分析伴有皮肤受累的青少年原发性干燥综合征（pSS）的临床特征。
我们调查了 37 例 pSS 患儿的临床和免疫学特征。所有患者均符合 2012 年美国风湿病学会干燥综合征分类标准。
19 例（不包括药物性皮疹）患儿出现皮肤受累，其中 16 例（84.2%）为女性，3 例（15.8%）为男性，平均年龄为 11±2.68 岁，其中 17 例（89.5%）以皮肤病变为首发症状，pSS 诊断前中位时间为 12 个月（1 天至 4 年）。皮肤病变包括 12 例可触及性紫癜（63.2%）、5 例荨麻疹（26.3%）、2 例皮肤干燥（10.5%）、1 例皮肤溃疡、1 例结节性红斑、1 例网状青斑、1 例雷诺现象和 1 例硬红斑。有皮肤病变的患儿关节受累发生率更高（42.1% vs. 11.1%，P=0.016）、发热（47.4% vs. 5.6%，P=0.004）、红细胞沉降率>50mm/h（47.4% vs. 11.1%，P=0.016）和血小板减少症（0% vs. 27.8%，P=0.013）及甲基强的松龙冲击治疗（0% vs. 13.5%，P=0.013）发生率较低。
半数以上（51.3%）的青少年 pSS 患儿出现皮肤病变；主要的皮肤受累是可触及性紫癜。有皮肤病变的患儿更易发热和关节炎，炎症反应更强，严重并发症更少。在许多情况下，皮肤病变可能是青少年 pSS 的首发症状，这容易导致误诊。对于出现可触及性紫癜和荨麻疹等皮肤病变的患儿，应考虑 pSS 的可能性，并进行进一步检查。
青少年 pSS 的皮肤病变并不少见，常表现为首发症状。
可触及性紫癜是青少年 pSS 最常见的皮肤病变，其次是荨麻疹。
伴有皮肤病变的青少年 pSS 不会增加血液系统损害等严重并发症的风险。
青少年 pSS 患者的皮肤病变可能容易导致误诊。
