Wieser Iris, Wohlmuth Christoph, Nunez Cesar A, Duvic Madeleine
Department of Dermatology, The University of Texas, MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030-4095, USA.
Department of Dermatology, Paracelsus Medical University, Salzburg, Austria.
Am J Clin Dermatol. 2016 Aug;17(4):319-27. doi: 10.1007/s40257-016-0192-6.
Lymphomatoid papulosis (LyP) is a lymphoproliferative disorder that is rare among adults and even rarer among children. In adults, LyP is associated with an increased risk of secondary lymphomas.
The aim of this systematic review was to describe the clinical and histopathological features of LyP in children, to assess the risk of associated lymphomas, and to compare the disease to the adult form.
A systematic review was conducted using the MEDLINE (PubMed), EMBASE, Scopus, and Cochrane databases from inception to June 2015. Articles were included if data were extractable from studies, case series, and single reports of pediatric LyP patients.
A total of 251 children and adolescents with LyP were identified, with the mean age at diagnosis being 9.3 ± 4.6 years (n = 187). The female to male ratio was 1:1.4, and the majority of children reported on were Caucasian (n = 74, 85.1 %). The predominant histologic subtype was type A (n = 106, 79.1 %). Clinically, LyP lesions presented as erythematous papules or nodules, appearing preferentially on the extremities and the trunk. LyP has to be differentiated from pityriasis lichenoides (PL) and primary cutaneous anaplastic large cell lymphoma (ALCL). PL and associated lymphomas were diagnosed before, with, and after LyP in 19 and 14 cases, respectively. Of the 14 subjects with associated lymphomas, two children developed systemic ALCL.
LyP has to be differentiated from ALCL to avoid erroneous treatments. Due to the increased risk of development of non-Hodgkin lymphomas, lifelong follow-up and proper patient counseling are warranted.
淋巴瘤样丘疹病（LyP）是一种淋巴增殖性疾病，在成人中罕见，在儿童中更为罕见。在成人中，LyP与继发性淋巴瘤风险增加相关。
本系统评价的目的是描述儿童LyP的临床和组织病理学特征，评估相关淋巴瘤的风险，并将该疾病与成人型进行比较。
使用MEDLINE（PubMed）、EMBASE、Scopus和Cochrane数据库进行系统评价，检索时间从建库至2015年6月。如果可从儿童LyP患者的研究、病例系列和单篇报告中提取数据，则纳入相关文章。
共确定了251例患有LyP的儿童和青少年，诊断时的平均年龄为9.3±4.6岁（n = 187）。男女比例为1:1.4，报告的大多数儿童为白种人（n = 74，85.1%）。主要组织学亚型为A型（n = 106，79.1%）。临床上，LyP皮损表现为红斑丘疹或结节，优先出现在四肢和躯干。LyP必须与苔藓样糠疹（PL）和原发性皮肤间变性大细胞淋巴瘤（ALCL）相鉴别。PL及相关淋巴瘤分别在LyP之前、同时和之后诊断出19例和14例。在14例伴有相关淋巴瘤的患者中，两名儿童发展为系统性ALCL。
LyP必须与ALCL相鉴别以避免错误治疗。由于非霍奇金淋巴瘤发生风险增加，有必要进行终身随访并给予患者适当的咨询。
