Clinicopathological features of Erdheim-Chester disease accompanied with Langerhans cell histiocytosis
Objective To report a case of Erdheim-Chester disease(ECD)as well Langerhans cell histiocytosis(LCH).Methods The diagnosis was confirmed by light microscopy,immunohistochemistry and BRAF V600E molecular detection,and relevant literature was reviewed.Results A 58-year-old woman with multiple skin masses and aggravation for 19 years was diagnosed with ECD.In this biopsy,we took a partial resection of the chest skin mass.Two components were observed under the microscope:one was foam histopathocytes with Dutton giant cells,and the other was Langerhans histopathocytes.In most areas,the two components were mixed.Immunohistochemical CD 163,CD68,Cyclin D1 were diffuse positive,CD1a,S-100,langerin partial positive;BRAF V600E mutation detected.After diagnosis,the skin masses of the whole body were significantly reduced after treatment with Tefilor for 1 month.Conclusion ECD combined with LCH is rare,and the diagnosis depends on imaging combined with morphological,immunohistochemical and molecular detection.The prognosis depends on the site of involvement.Patients with bone and skin involvement alone may have a relatively good prognosis,and patients with BRAF V600E mutations may benefit significantly from targeted therapy.
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