Effendi Raden Mohamad Rendy Ariezal, Rizqandaru Trustia, Yuliasari Renata, Gondokaryono Srie Prihianti, Diana Inne Arline, Dwiyana Reiva Farah
Department of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Bandung, Indonesia.
Clin Cosmet Investig Dermatol. 2022 Aug 6;15:1575-1582. doi: 10.2147/CCID.S375995. eCollection 2022.
Non-Langerhans cell histiocytosis (non-LCH) is a group of diseases characterized by the proliferation of histiocytes in tissues that is excluded from the diagnostic criteria for LCH. Juvenile xanthogranuloma (JXG) and benign cephalic histiocytosis (BCH) are the most common types of cutaneous non-LCH. These two diseases share similarities in both clinical and histological features, therefore, they can be difficult to differentiate. Thorough physical, dermoscopic, and histopathological examinations are required to distinguish between JXG and BCH. We hereby present two rare cases of non-LCH in pediatric patients, presented with JXG and BCH. The dermoscopic examination of both cases showed a setting-sun appearance, while the histopathological examination revealed Touton giant cells in the JXG case, and massive lymphocyte infiltration in the BCH case. Both patients were treated with 1% topical rapamycin in a split-side comparison for the first 12 weeks, followed by applications on both sides for a total duration of 24 weeks. As a result, there was a significant reduction in the size of the lesion, leading to patient's satisfaction. Rapamycin is an immunosuppressive agent with antineoplastic activity. Rapamycin can be used as an alternative non-invasive topical treatment option for JXG and BCH. However, long-term observations are required to assess its effectiveness and side effects.
非朗格汉斯细胞组织细胞增生症（non-LCH）是一组以组织中组织细胞增生为特征的疾病，其被排除在LCH的诊断标准之外。幼年性黄色肉芽肿（JXG）和良性头部组织细胞增生症（BCH）是皮肤非-LCH最常见的类型。这两种疾病在临床和组织学特征上都有相似之处，因此，它们可能难以区分。需要进行全面的体格检查、皮肤镜检查和组织病理学检查来区分JXG和BCH。我们在此报告两例儿科患者的罕见非-LCH病例，分别表现为JXG和BCH。两例病例的皮肤镜检查均显示落日征，而组织病理学检查显示JXG病例有 Touton 巨细胞，BCH病例有大量淋巴细胞浸润。两名患者在最初12周采用1%外用雷帕霉素进行双侧对比治疗，随后双侧应用，总疗程为24周。结果，病变大小显著减小，患者满意。雷帕霉素是一种具有抗肿瘤活性的免疫抑制剂。雷帕霉素可作为JXG和BCH的一种替代性非侵入性局部治疗选择。然而，需要长期观察来评估其有效性和副作用。
