孤立性眼眶朗格汉斯细胞组织细胞增多症的非典型表现。
Atypical presentation of isolated orbital Langerhans cell histiocytosis.
作者信息
Richards Nikisha Q, Young Matthew, Pierson Kasey, Le John, Rong Yuan
机构信息
Department of Ophthalmology, Virginia Commonwealth University, Richmond, VA.
Department of Pathology, Virginia Commonwealth University, Richmond, VA.
出版信息
Sarcoidosis Vasc Diffuse Lung Dis. 2019;36(2):167-171. doi: 10.36141/svdld.v36i2.7639. Epub 2019 May 1.
BACKGROUND
A 9-year old female presented with one month of waxing and waning upper eyelid swelling. An excisional biopsy via anterior orbitotomy was performed.
OBJECTIVE
To describe a patient presenting atypically with symptoms concerning for orbital cellulitis who was diagnosed with Langerhans cell histiocytosis (LCH).
METHODS
Description of case report.
RESULTS
We report a case of a 9-year old female with one month of periorbital edema and erythema suspected to be orbital cellulitis. A complete ophthalmological exam, subsequent imaging, and an excisional biopsy revealed the diagnosis of LCH. With a confirmed diagnosis, the patient started chemotherapy indicated by the Histiocyte Society Evaluation and Treatment Guidelines.
CONCLUSION
Langerhans cell histiocytosis (LCH) embodies a spectrum of diseases with the primary pathologic process being the abnormal proliferation of polyclonal Langerhans cells. In children with isolated bony involvement, the most common presenting symptom is pain. Rarely is orbital involvement with associated periorbital edema and erythema the primary presentation.
背景
一名9岁女性出现上睑肿胀，时轻时重，持续1个月。通过前路眶切开术进行了切除活检。
目的
描述一名以疑似眼眶蜂窝织炎的非典型症状就诊，最终被诊断为朗格汉斯细胞组织细胞增多症（LCH）的患者。
方法
病例报告描述。
结果
我们报告一例9岁女性，有1个月的眶周水肿和红斑，疑似眼眶蜂窝织炎。完整的眼科检查、后续影像学检查及切除活检确诊为LCH。确诊后，患者开始按照组织细胞协会评估和治疗指南进行化疗。
结论
朗格汉斯细胞组织细胞增多症（LCH）涵盖一系列疾病，主要病理过程是多克隆朗格汉斯细胞的异常增殖。在孤立性骨受累的儿童中，最常见的症状是疼痛。眼眶受累伴眶周水肿和红斑作为主要表现的情况很少见。
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