文献简介

出版社:JAMA Dermatology

作  者:Stephanie Nagel, MD; Hagen Graf Einsiedel, MD; Manfred Kunz, MD

编  号:

关键字:多系统性朗格汉斯细胞组织细胞增多症

年  份:2018   点击量:264

文献摘要 全文翻译

A 4-year-old boy presented to our department of dermatology with a 3-year history of nonhealing crusted scalp lesions. He had previously been treated with various topical glucocorticosteroids and antibiotics without improvement. The physical examination revealed multiple disseminated erythematous papules, petechial hemorrhages, and yellowish crusts on the scalp (Figure, A). The remaining skin and mucosal surfaces, as well as neck, axillary, and inguinal lymph nodes, were unremarkable. The patient did not show any other obvious abnormalities, but his mother reported occasional otitis media. The family history was negative for skin disease. Routine blood examinations revealed no pathological findings. A whole-body magnetic resonance imaging (MRI) scan showed 2 osteolytic lesions on the right frontoparietal skull (Figure, B). A punch biopsy specimen of an erythematous papule on the scalp was obtained and stained with hematoxylin-eosin for histopathological analysis (Figure, C). In addition, immunohistochemical stainings were performed (Figure, D).

患者男,4岁,就诊皮肤科,有3年不愈合痂皮鳞屑皮损史。患者曾采用各种局部糖皮质激素和抗生素治疗,但皮损无改善。体格检查示头皮可见多个播散性红斑丘疹、点状出血和黄色痂皮(图A)。其余皮肤和粘膜表面,以及颈部、腋窝、腹股沟淋巴结处均不明显。患者无任何其他明显异常,但其母称有偶发性中耳炎。患者无类似皮肤病家族史。常规血液检查无病理学发现。全身磁共振成像(MRI)扫描显示右侧额顶颅骨存在2个溶骨性病变(图B)。在皮肤红斑丘疹处取钻孔活检标本,并进行HE染色用于进行组织病理学分析(图C)。此外,行免疫组化染色(图D)。