背景:淋巴瘤样丘疹病(LyP)是一种罕见的皮肤T细胞淋巴增生性疾病。关于儿科人群LyP的全面数据很少。
目的:为了描述流行病学特征,临床,组织病理学,儿科LyP的预后特征。
方法:这是一个回顾性研究,多中心国际队列研究,包括1998年至2022年间诊断为LyP的87例儿童和青少年。包括发病时年龄≤18岁的患者。根据临床病理相关性在每个中心进行诊断。
结果:纳入了来自12个中心的87例患者。发病时的平均年龄为7.0岁(3个月-18岁),男女比例为2:1。首次皮肤病变发作与诊断之间的平均时间为1.3年(范围为0-14年)。最初误诊的患者占26.4%。最初,LyP最常被误诊为地衣糠疹和天花糠疹(PLEVA),昆虫叮咬,或者传染性软体动物.红斑丘疹或丘疹结节是最常见的临床表现。20.7%的患者特别提到瘙痒。55.1%的病例主要组织学亚型为A型。如果分析,在76.5%的皮肤活检中发现了单克隆TCR重排。33例患者的5年随访和8例患者的15年随访,总生存率为100%。在9.6%的病例中发生了相关的血液恶性肿瘤(HM)的发展(7/73),包括4例支原体(MF)病例,原发性皮肤间变性大细胞淋巴瘤(pc-ALCL),1例全身性ALCL和1例急性髓系白血病。如果我们比较2001-2010年世界0-19岁人群的癌症发病率,我们估计总体上相关恶性肿瘤的风险明显更高,发生在19岁之前,发生率为87.49(CI86.01-88.99)。
结论:我们报告了来自大型国际儿童和青少年LyP患者队列的流行病学数据。总体来说,这种疾病的预后是好的,所有患者的生存率都很高。由于相关HM的风险增加,建议对LyP患者进行长期随访.
BACKGROUND: Lymphomatoid papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population are scarce.
OBJECTIVE: To characterize the epidemiological, clinical, histopathological and prognostic features of paediatric LyP.
METHODS: This was a retrospective multicentre international cohort study that included 87 children and adolescents with LyP diagnosed between 1998 and 2022. Patients aged ≤ 18 years at disease onset were included. LyP diagnosis was made in each centre, based on clinicopathological correlation.
RESULTS: Eighty-seven patients from 12 centres were included. Mean age at disease onset was 7.0 years (range 3 months-18 years) with a male to female ratio of 2 : 1. Mean time between the onset of the first cutaneous lesions and diagnosis was 1.3 years (range 0-14). Initial misdiagnosis concerned 26% of patients. LyP was most often misdiagnosed as pityriasis lichenoides et varioliformis acuta, insect bites or mollusca contagiosa. Erythematous papules or papulonodules were the most frequent clinical presentation. Pruritus was specifically mentioned in 21% of patients. The main histological subtype was type A in 55% of cases. When analysed, monoclonal T-cell receptor rearrangement was found in 77% of skin biopsies. The overall survival rate was 100%, with follow-up at 5 years available for 33 patients and at 15 years for 8 patients. Associated haematological malignancy (HM) occurred in 10% of cases (n = 7/73), including four patients with mycosis fungoides, one with primary cutaneous anaplastic large cell lymphoma (ALCL), one with systemic ALCL and one with acute myeloid leukaemia. If we compared incidence rates of cancer with the world population aged 0-19 years from 2001 to 2010, we estimated a significantly higher risk of associated malignancy in general, occurring before the age of 19 years (incidence rate ratio 87.49, 95% confidence interval 86.01-88.99).
CONCLUSIONS: We report epidemiological data from a large international cohort of children and adolescents with LyP. Overall, the disease prognosis is good, with excellent survival rates for all patients. Owing to an increased risk of associated HM, long-term follow-up should be recommended for patients with LyP.
Lymphomatoid papulosis is a very rare skin condition caused by an abnormal increase in white blood cells (called ‘lymphocytes’) in the skin. The condition rarely affects children, so most of the scientific data published about this disease focuses on adults. This study involved 12 academic dermatology centres in Europe, the Middle East and North America, and gathered data from about 87 children who presented with symptoms of lymphomatoid papulosis before the age of 19 years. The aim of this study was to better describe this disease in the paediatric population and discuss its treatment options and evolution. We found that the presentation of the disease in children is roughly the same as in adults. Safe and effective treatment options exist. The disease is not life threatening, but it requires investigation by a dermatologist, both to make a careful diagnosis and to monitor it as sometimes associated cancers that originate from blood cells can occur, mostly on the skin.