Primary Sjogren′s syndrome in children with rash as the first symptom: a case report and literature review

doi:10.3877/cma.j.issn.1673-5250.2020.06.009

Objective

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

Methods

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.

Results

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.

Conclusions

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.

Key words: Sjogren′s syndrome, Exanthema, Exocrinegland, SS-A antigen, SS-B antigen, Labial gland biopsy, Rheumatoid factor, Early diagnosis, Child

图1 本例原发性干燥综合征患儿(女性，13岁11个月)左上臂皮疹形态[皮疹为水肿性红斑，向周围扩大呈环状或弧状，外边缘呈鲜红色并且水肿样隆起，最长径(AB)为10 cm]

图2 本例原发性干燥综合征患儿(女性，13岁11个月)腮腺增强CT图像[左侧充盈相示腮腺稍增大，腮腺导管造影分布不均，散在微小囊状扩张导管(白色箭头所示)]

图3 本例原发性干燥综合征患儿(女性，13岁11个月)唇腺组织病理学图(HE染色，低倍)[涎腺组织的小叶结构存在，小叶内淋巴细胞浸润，每个聚集灶(红色箭头所示)内淋巴细胞>50个]

表1 本例pSS患儿治疗前、后血常规主要指标及CRP水平检测结果

检查时间(年-月-日)	白细胞计数(×10⁹/L)	红细胞计数(×10¹²/L)	血红蛋白水平(g/L)	血小板计数(×10⁹/L)	中性粒细胞百分比(%)	淋巴细胞百分比(%)	CRP水平(mg/L)
2019-07-30	12.73	4.75	136	241	88.0	6.8	-
2019-08-02	6.35	4.60	128	249	65.0	26.1	16.0
2019-08-05	3.87	4.80	136	288	55.5	34.9	2.0
2019-08-08	4.82	4.84	135	327	58.7	32.8	1.0
2019-08-11	7.93	4.63	130	367	58.0	32.5	<1.0
正常参考值	4.00～10.00	3.80～5.10	110～155	100～450	50.0～70.0	20.0～40.0	0～10.0

表1 本例pSS患儿治疗前、后血常规主要指标及CRP水平检测结果

检查时间(年-月-日)	白细胞计数(×10⁹/L)	红细胞计数(×10¹²/L)	血红蛋白水平(g/L)	血小板计数(×10⁹/L)	中性粒细胞百分比(%)	淋巴细胞百分比(%)	CRP水平(mg/L)
2019-07-30	12.73	4.75	136	241	88.0	6.8	-
2019-08-02	6.35	4.60	128	249	65.0	26.1	16.0
2019-08-05	3.87	4.80	136	288	55.5	34.9	2.0
2019-08-08	4.82	4.84	135	327	58.7	32.8	1.0
2019-08-11	7.93	4.63	130	367	58.0	32.5	<1.0
正常参考值	4.00～10.00	3.80～5.10	110～155	100～450	50.0～70.0	20.0～40.0	0～10.0

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