探讨以皮疹为首发症状的儿童原发性干燥综合征(pSS)的临床诊治要点。

选择2019年8月2日，成都市妇女儿童中心医院收治的1例以皮疹为首发症状的pSS患儿为研究对象。回顾性分析其临床病例资料，并对2000年1月至2020年4月的国内外数据库中，关于儿童pSS的相关文献进行复习。本研究符合2013年修订的《世界医学协会赫尔辛基宣言》要求。

本例pSS患儿为女性，年龄为13岁11个月，因主诉"皮疹3^＋年，加重4个月"收入本院。①临床表现：皮疹位于患儿颜面部及双上肢，呈红色水肿性红斑，并部分融合；伴脱发、发热、右眼结合膜充血，不伴眼干、口干、皮肤干燥及腮腺肿大等临床表现。②主要实验室检查结果：血清类风湿因子(RF)水平升高，抗核抗体(ANA)免疫球蛋白(Ig)G呈阳性，抗干燥综合征(SS)A、B抗体均呈阳性；双眼角膜荧光染色(＋)，唇腺活组织检查示腺体中淋巴细胞密集性聚集、局灶性淋巴细胞浸润改变，腮腺增强CT检查结果显示双侧腮腺稍增大、密度欠均匀、散在微小囊状扩张导管。③治疗及转归：免疫调节及免疫抑制治疗，以及局部(眼)给予滴眼液外用后，皮疹及眼部症状均好转，出院后继续上述治疗，病情基本痊愈。对其随访至14岁8个月时，疾病无复发。pSS文献复习结果：儿童pSS临床表现多样，确诊该病的检查较复杂；对儿童pSS目前尚无特异性治疗方法，主要为系统性治疗配合局部治疗。

以皮疹为首发症状的儿童pSS较罕见，血清学抗体检测、外分泌腺体组织病理学与影像学检查，有助于该病患儿的早期诊断。

To explore clinical diagnosis and treatment of primary Sjogren′s syndrome (pSS) in children with rash as the first symptom.

A case of pSS with rash as the first symptom who admitted to Chengdu Women′s and Children′s Central Hospital on 2 August, 2019 was selected as research subject.Her clinical case data was analyzed retrospectively. The related literature of pSS in children was reviewed. This study met requirements of World Medical Association Declaration of Helsinki revised in 2013.

This girl was 13 years and 11 months, as suffered from " rash for 3⁺ years, aggravated for 4 months" when admitted to hospital. ① Clinical manifestations: rash around her face and upper limbs were red with edematous erythema, and partial fusion.She was accompanied with alopecia, fever and conjunctival hyperemia in the right eye. There were no clinical manifestations such as dry eyes, dry mouth, dry skin and swelling of parotid gland. ② Main laboratory examination results: serum rheumatoid factor (RF) level increased, antinuclear antibody (ANA) immunoglobulin (Ig) G was positive, anti-Sjogren′s syndrome (SS) A and B antibody both were positive; fluorescence staining (+ ) in cornea of both eyes, biopsy of lip gland showed that lymphocytes were densely gathered and focal lymphocyte infiltration, enhanced CT examination of parotid gland showed that bilateral parotid glands were slightly enlarged, with uneven density, scattered in tiny saccular dilatation catheter. ③Treatment and sequelae: skin rash and eye symptoms were improved after immunomodulation and immunosuppression treatment, and topical external application with eye drops. She continued above treatment after discharge and the disease basically recovered. She was follow-up to 14 years and 8 months old without recurrence. Results of literature review: children′s pSS have various clinical manifestations and examination for diagnosis of pSS were complicated. There was no specific treatment for children′s pSS at present, and systematic treatment combined with local treatment was the main treatment.

Children′s pSS with rash as the first symptom is rare, and serological antibody detection, exocrine gland histopathology and imaging examination are helpful for early diagnosis of children with pSS.