Department of Dermatology, Eastern Virginia Medical School, 721 Fairfax Ave, Suite 200, Norfolk, VA 23507, USA.
Pediatrics. 2011 Jan;127(1):e226-30. doi: 10.1542/peds.2009-2019. Epub 2010 Dec 6.
Hemorrhagic vesicles in a newborn present a challenging differential diagnosis including both infectious and neoplastic disorders. Patients should be evaluated in an efficient manner to arrive at the correct diagnosis as quickly as possible. We present here an interesting case that outlines the methodical workup that ultimately revealed the diagnosis of congenital Langerhans cell histiocytosis. After a diagnosis of Langerhans cell histiocytosis is made, it is important to evaluate the patient thoroughly for systemic involvement. Historically, the diagnosis of congenital self-healing Langerhans cell histiocytosis was used to delineate a benign self-limited disorder limited to the skin with spontaneous resolution during the first several months of life; this disorder may also be referred to as "self-regressive Langerhans cell histiocytosis." However, some newborns with initial skin-only Langerhans cell histiocytosis progress to have multisystem disease after spontaneous resolution has occurred. For this reason, the nomenclature is changing. We suggest using the term "skin-only Langerhans cell histiocytosis." Periodic long-term follow-up is recommended to monitor for relapse or progression to systemic disease.
新生儿出现出血性水疱，其鉴别诊断具有挑战性，包括感染性和肿瘤性疾病。应尽快以有效的方式对患者进行评估，以便尽快得出正确的诊断。我们在此介绍一个有趣的病例，该病例概述了最终揭示先天性朗格汉斯细胞组织细胞增生症诊断的系统检查方法。朗格汉斯细胞组织细胞增生症诊断后，重要的是要彻底评估患者是否存在全身受累。从历史上看，先天性自愈性朗格汉斯细胞组织细胞增生症的诊断用于描述一种良性、自限性疾病，局限于皮肤，在生命的头几个月内自发消退；这种疾病也可能被称为“自限性朗格汉斯细胞组织细胞增生症”。然而，一些最初仅表现为皮肤朗格汉斯细胞组织细胞增生症的新生儿在自发消退后会进展为多系统疾病。因此，命名法正在发生变化。我们建议使用“仅皮肤朗格汉斯细胞组织细胞增生症”一词。建议进行定期的长期随访，以监测复发或进展为全身疾病。
