背景:淋巴瘤样丘疹病(LyP)是儿科罕见的疾病;仅有7名儿童报道了LyP组织学类型D。LyP在淋巴增生谱中的鉴别诊断仍存在争议。
方法:一名6岁男孩到急诊科就诊,有3周的下颌下区域出现红斑丘疹-水疱瘙痒性皮疹,躯干和四肢。历史,症状和实验室检查不明显。SARS-CoV-2抗原阴性。临床上怀疑是地衣糠疹和天花糠疹(PLEVA),并引入了局部类固醇。一周后,他带着大面积疼痛的鳞状丘疹-红斑皮疹回来,有一些溃疡和坏死性病变,和发烧;因此孩子住院了。生化结果在参考范围内,除了高水平的C反应蛋白,天冬氨酸转氨酶,丙氨酸转氨酶和胆红素。由于持续的高烧,全身皮质类固醇治疗,具有良好的临床反应和皮肤病变的改善。检测到抗PVB-19免疫球蛋白M。还记录了升高的IL-6、IL-10和IFN-γ水平。入院后五天,大部分的病灶已经清除,孩子出院了.开始使用甲氨蝶呤,以积极的回应。在皮肤活检中,“PLEVA样”模式很明显,有一个密集的,楔形淋巴样浸润,具有表皮性,形态上包括多形性和母细胞。免疫组织化学染色突出了浸润的模式,这证明了该过程具有CD8+/CD30+表型,后者在较大的细胞上强烈,抗原性丧失。用于T细胞受体γ(TCRG)链克隆性评估的聚合酶链反应记录了单克隆峰。诊断为D型LyP是有利的。
结论:报告的病例包含了两个独立实体的大部分关键特征——PLEVA和LyP——因此为它们代表唯一疾病谱内的下降的概念提供了进一步的支持。研究感染因子在淋巴增生性皮肤疾病中作为触发潜力的作用,并检测新的疾病标志物,如细胞因子,可能对致病性疾病机制和观点治疗产生至关重要的影响。
BACKGROUND: Lymphomatoid papulosis (LyP) is a rare condition in pediatrics; LyP histological type D has been reported in only 7 children. The differential diagnosis of LyP in the spectrum of lymphoid proliferation remains controversial.
METHODS: A 6-year-old boy presented to Emergency Department with a 3-week history of an erythematous papulo-vesicular itchy eruption over the submandibular regions, trunk and extremities. History, symptoms and laboratory tests were unremarkable. SARS-CoV-2 antigen was negative. The clinical suspicion of pityriasis lichenoides et varioliformis acuta (PLEVA) was posed, and topical steroids were introduced. One week after, he returned with an extensive painful scaly papulo-erythematous rash, with some ulcerated and necrotic lesions, and fever; therefore the child was hospitalized. Biochemical results were within reference limits, except for high level of C-reactive protein, aspartate aminotransferase, alanine transaminase and bilirubin. Due to a persistently high fever, systemic corticosteroid treatment was administered, with a good clinical response and an improvement of the skin lesions. Anti-PVB-19 Immunoglobulin M was detected. Elevated levels of IL-6, IL-10 and IFN-γ were also recorded. Five days post-admission, most of the lesions had cleared, and the child was discharged. Methotrexate was started, with a positive response. At skin biopsy a \"PLEVA-like\" pattern was apparent, with a dense, wedge shaped lymphoid infiltrate featuring epidermotropism and morphologically comprising pleomorphic and blastic cells. The pattern of infiltration was highlighted by immunohistochemical stains, which prove the process to feature a CD8+/CD30 + phenotype, the latter being intense on larger cells, with antigenic loss. Polymerase chain reaction for T-cell receptor gamma (TCRG) chain clonality assessment documented a monoclonal peak. A diagnosis of LyP type D was favored.
CONCLUSIONS: The reported
case encompasses most of the critical features of two separated entities-PLEVA and LyP-thus providing further support to the concept of them representing declinations within a sole spectrum of disease. Studying the role of infectious agents as trigger potential in lymphoproliferative cutaneous disorders and detecting novel markers of disease, such as cytokines, could have a crucial impact on pathogenic disease mechanisms and perspective therapies.