Clinical case of successful treatment of continuously recurrent Langerhans cell histiocytosis with BRAF-inhibitor

Nowadays, treatment results of Langerhans cell Histiocytosis (LCH) are successful. Modern treatment protocols LCH III, LCH IV can reach 100% survival in patients with single-system LCH. In case of multisystem disease, 5-year overall survival (OS) is rated at 81,2%. Relapses should be treated with more intensive polyshemotherapy, which is working (5-year OS in relapses reach 85%), but highly toxic. One possibility in LCH relapses treatment is targeted therapy of gene BRAF mutation, that detected in 60% of patients with LCH. BRAF-inhibitors just came into use in pediatric LCH practice, but preliminary results hold out a hope of high efficacy and low toxicity. In the current issue a difficult differential, diagnostic, and therapeutic clinical case of LCH in a 4-year patient is presented. Onset of disease was as skin lesions, that became a reason of mistaken diagnosis of atopic dermatitis, ineffective treatment with topical corticosteroids, joined systemic signs throw discredit upon diagnosis and perform a skin biopsy. At the time of morpho-immunological diagnosis LCH disseminated as multisystem, multifocal disease, that progressed on first- and second-line therapy. Trying to treat with BRAFinhibitor (vemurafenib) became successful. A partial effect was achieved.