Mendonça-Sanches Maria, Rolim Inês, Costa-Rosa Joaninha, Caldas Gabriela, Moura Cecília
Dermatology Department, Hospital de Santa Maria, Lisbon, Portugal.
Pathology Department, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal.
Eur J Case Rep Intern Med. 2019 Jun 5;6(6):001088. doi: 10.12890/2019_001088. eCollection 2019.
Langerhans cell histiocytosis (LCH) is a multisystemic disorder that results from the clonal proliferation of immunophenotypically and functionally immature Langerhans cells (LC). The detection of the V600E mutation in the BRAF oncogene in LCH biopsy specimens supports previous evidence that LCH is a neoplastic disorder. This mutation is present in other cutaneous lesions including malignant melanoma and benign nevi. Single case reports of a correlation between LCH and the appearance of eruptive nevi limited to the inguinal folds after chemotherapy have previously been described in the literature. This suggested that LCH could be an additional cause of eruptive melanocytic nevi, with a specific distribution mimicking that of LCH cutaneous lesions. We present the case of a 6-year-old boy, previously treated with chemotherapy for Langerhans cell histiocytosis, with disseminated junctional nevi. Although this co-occurrence may be coincidental, the skin involvement is distinct from other previously reported clinical cases. It would be interesting to evaluate whether the BRAF mutation described in LCH cells might in fact support a genetic background for the development of nevi in these patients.
Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of immature Langerhans cells, with the V600E mutation in the BRAF oncogene present in approximately 60% of cases.The V600E mutation in the BRAF oncogene is also documented in other cutaneous lesions, namely malignant melanoma and benign nevi.There are case reports of a correlation between LCH and the appearance of eruptive nevi after chemotherapy, but it is not known whether the BRAF mutation described in LCH cells supports a genetic background for the development of nevi in these patients.
朗格汉斯细胞组织细胞增多症（LCH）是一种多系统疾病，由免疫表型和功能不成熟的朗格汉斯细胞（LC）克隆性增殖引起。LCH活检标本中BRAF癌基因V600E突变的检测支持了先前关于LCH是一种肿瘤性疾病的证据。该突变也存在于其他皮肤病变中，包括恶性黑色素瘤和良性痣。先前文献中曾有单例报道描述了LCH与化疗后腹股沟皱襞处出现的发疹性痣之间的相关性。这表明LCH可能是发疹性黑素细胞痣的另一个病因，其特定分布与LCH皮肤病变相似。我们报告一例6岁男孩，曾因朗格汉斯细胞组织细胞增多症接受化疗，出现播散性交界痣。尽管这种同时出现可能是巧合，但皮肤受累情况与其他先前报道的临床病例不同。评估LCH细胞中描述的BRAF突变是否实际上支持这些患者痣形成的遗传背景将是很有趣的。
朗格汉斯细胞组织细胞增多症（LCH）是不成熟朗格汉斯细胞的克隆性肿瘤性增殖，约60%的病例存在BRAF癌基因的V600E突变。BRAF癌基因的V600E突变也见于其他皮肤病变，即恶性黑色素瘤和良性痣。有病例报告称LCH与化疗后发疹性痣的出现有关，但尚不清楚LCH细胞中描述的BRAF突变是否支持这些患者痣形成的遗传背景。
