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Mikulicz Disease.
JAMA Dermatology ( IF 10.9 ) Pub Date : 2020-04-01 , DOI: 10.1001/jamadermatol.2019.4260
Chao Wu 1 , Yue-Ping Zeng 1
Affiliation  

A man in his 60s had a 12-year history of bilateral palpebral swelling. He also had dryness of the eyes and mouth. A physical examination showed bilateral swelling of the upper and lower eyelids (Figure). Spindle-shaped subcutaneous masses were found by palpation of the eyelids. Results of laboratory tests showed high levels of serum IgG4 (1240 mg/dL; to convert to grams per liter, multiply by 0.01) and IgE (401 μg/L; to convert to milligrams per liter, multiply by 0.001). Serum levels of high-sensitivity C-reactive protein (10.08 mg/L; to convert to nanomoles per liter, multiply by 9.524) and erythrocyte sedimentation rate (30 mm/h) were both elevated. Test results were negative for serum antinuclear antibodies and extractable nuclear antigens.Magnetic resonance imaging revealed several hypoechoic lymph nodes in the right parotid gland and submandibular region and diffuse lesions involving the bilateral orbits, eyelids, lacrimal glands, and subcutaneous tissues of the temporal regions. Cutaneous biopsy results from the right upper eyelid were nondiagnostic. Histopathologic examination of the left lacrimal gland revealed a massive infiltration of lymphocytes and plasma cells. Immunostaining showed infiltration of IgG4-positive cells. A diagnosis of Mikulicz disease was made. The patient was treated with 30 mg/d of oral prednisolone, which was tapered for 2 months. The patient had partial remission of the palpebral swelling at last follow-up.



中文翻译:

米库利兹病。

一名60多岁的男子有12年的双侧睑板肿胀史。他的眼睛和嘴巴也干燥。身体检查显示上下眼睑双侧肿胀(图)。通过触诊眼睑发现纺锤形的皮下肿物。实验室测试结果显示高水平的血清IgG4(1240 mg / dL;换算成克/升,乘以0.01)和IgE(401μg/ L;换算成毫克/升,乘以0.001)。高敏C反应蛋白的血清水平(10.08 mg / L;转换为每升纳摩尔,乘以9.524)和红细胞沉降速率(30 mm / h)均升高。血清抗核抗体和可提取的核抗原测试结果均为阴性。磁共振成像显示右腮腺和下颌下区域有几个低回声淋巴结,弥散性病变累及双侧眼眶,眼睑,泪腺和颞区的皮下组织。右上眼皮的皮肤活检结果无法诊断。左泪腺的组织病理学检查显示淋巴细胞和浆细胞大量浸润。免疫染色显示IgG4阳性细胞浸润。诊断为米库利奇病。该患者接受了30 mg / d的泼尼松龙口服治疗,逐渐减量2个月。在最后一次随访中,患者的睑睑肿胀得到部分缓解。右上眼皮的皮肤活检结果无法诊断。左泪腺的组织病理学检查显示淋巴细胞和浆细胞大量浸润。免疫染色显示IgG4阳性细胞浸润。诊断为米库利奇病。该患者接受了30 mg / d的泼尼松龙口服治疗,逐渐减量2个月。在最后一次随访中,患者的睑睑肿胀得到部分缓解。右上眼皮的皮肤活检结果无法诊断。左泪腺的组织病理学检查显示淋巴细胞和浆细胞大量浸润。免疫染色显示IgG4阳性细胞浸润。诊断为米库利奇病。该患者接受了30 mg / d的泼尼松龙口服治疗,逐渐减量2个月。在最后一次随访中,患者的睑睑肿胀得到部分缓解。

更新日期:2020-04-01
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