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Eruptive Junctional Nevi Appearing During Langerhans Cell Histiocytosis Treatment
Maria Mendonça-Sanches1, Inês Rolim2, Joaninha Costa-Rosa2
1Dermatology Department, Hospital de Santa Maria, Lisbon, Portugal.
Insights
Langerhans cell histiocytosis (LCH) may cause eruptive nevi, potentially linked to the BRAF V600E mutation found in both LCH and nevi. Further research is needed to confirm if this mutation provides a genetic basis for nevi development in LCH patients.
Area of Science:
- Oncology
- Dermatology
- Genetics
Background:
- Langerhans cell histiocytosis (LCH) is a neoplastic disorder involving clonal proliferation of Langerhans cells.
- The V600E mutation in the BRAF oncogene is detected in LCH, supporting its neoplastic nature.
- This BRAF mutation is also found in cutaneous lesions like melanoma and nevi.
Purpose of the Study:
- To investigate a potential correlation between Langerhans cell histiocytosis (LCH) and the development of eruptive melanocytic nevi.
- To explore if the BRAF V600E mutation in LCH could predispose patients to developing nevi.
Main Methods:
- Case report of a 6-year-old boy treated for LCH.
- Observation of disseminated junctional nevi following chemotherapy for LCH.
- Review of existing literature on LCH and eruptive nevi.
Main Results:
- The patient presented with disseminated junctional nevi after chemotherapy for LCH.
- The distribution of nevi was distinct from previously reported cases.
- The V600E BRAF mutation is common in LCH and present in nevi.
Conclusions:
- Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation with a high prevalence of the BRAF V600E mutation.
- The co-occurrence of LCH and eruptive nevi suggests a possible link, potentially mediated by the BRAF mutation.
- Further investigation is warranted to determine if the BRAF mutation in LCH provides a genetic predisposition for nevus development.
Abstract:
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.
Learning Points:
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.
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