Abstract
Langerhans Cell Histiocytosis (LCH) is a rare disease with a variable clinical presentation and prognosis. The authors present a case report of 11month old boy with LCH.
The patient had skin symptoms from his birth that were considered to be a neonatal toxoalergic rash, and then subsequently, to be a seborhoic dermatitis, atopic eczema and perianal intertrigo. He suffered from recurrent respiratory tract infections and since the age of six months, he had secretion from his both ears, treated as an external otitis.
Shortly before the correct diagnosis of LCH was assigned, the patient developed anemia, trombocytopenia and neutropenia. The multi-system LCH also affected other risk organs - liver and spleen.The therapy began with vinblastine and prednisolone according to the international protocol for treatment of LCH.
The disease did not responed to standard induction therapy, so an experimental treatment with BRAF inhibitor was commenced with a prompt response and regression of symptoms. The article focuses on the description of symptoms of LCH with an aim to raise awareness of the disease, to prevent delayed diagnosis, which can happen due to confusion of symptoms of other more common diseases.