Extracellular signal‐regulated kinase activation of self‐healing Langerhans cell histiocytosis: A case report. Issue 9 (4th July 2019)

• Record Type: Journal Article
• Authors: Kaneshima, Akiko; Yamaguchi, Sayaka; Miyagi, Takuya; Kariya, Yoshiyuki; Awazawa, Tsuyoshi; Ohshiro, Tokiko; Hyakuna, Nobuyuki; Nakanishi, Koichi; Takahashi, Kenzo
• Is Part Of: Journal of dermatology. Volume 46:Issue 9(2019)
• Page Start: 812
• Page End: 815
• Publication Date: 2019-07-04
• Subjects: BRAF V600E -- extracellular signal‐regulated kinase activation -- Hashimoto–Pritzker self‐healing reticulohistiocytosis -- Langerhans cell histiocytosis -- self‐healing Langerhans cell histiocytosis; Dermatology -- Periodicals; Dermatology -- Japan -- Periodicals; Skin -- Diseases -- Periodicals; 616.5005
• DOI: 10.1111/1346-8138.14996
• Languages: English
• ISSNs: 0385-2407

Abstract

Abstract: A 3‐month‐old boy developed small papules on his trunk. After the papules increased in number, the patient was diagnosed with Langerhans cell histiocytosis based on the pathological findings. He was referred to our department for further examination. Upon initial examination, the papules and nodules were scattered on his back, abdomen and lumbar region. Because he did not present with any organ involvement except the skin, he was diagnosed with single‐system and skin‐limited Langerhans cell histiocytosis. Skin rashes were treated with a topical steroid and started regressing 3 months after onset. All papules disappeared 6 months after onset. In this boy, the Langerhans cell histiocytosis tumor cells expressed phosphorylated extracellular signal‐regulated kinases. In Langerhans cell histiocytosis, BRAF V600E and other genes are known to mutate to act as driver mutations in stem cells of the myeloid dendritic cell lineage. Consequently, extracellular signal‐regulated kinases are continuously activated, which contributes to Langerhans cell histiocytosis carcinogenesis.