Abstract

Histiocytosis are heterogeneous group of diseases characterized by the proliferation of cells of the mononuclear phagocytic system (monocytes, macrophages, dendritic cells) in different tissues, they are infrequent entities, predominantly in childhood, with variable severity. Langerhans cell histiocytosis (LCH) is a reactive process of unknown etiology, with heterogeneous clinical manifestations and variable prognosis determined by the age of onset and organ involvement. We present the case of a term newborn with no relevant family or obstetric history, who presented generalized skin lesions with palmoplantar involvement at the oral cavity from birth. A skin biopsy was performed, with histopathological and immunohistochemical findings compatible with the diagnosis of congenital Langerhans cell histiocytosis. For being a unisystemic disease, due to skin involvement, with special site involvement of hard palate, according to the classification of the Histiocytosis Society, it was considered a candidate to management with chemotherapy by HCL - INC-2012 protocol, based on vinblastine and prednisolone. The patient completed the first cycle of the initial treatment, with complete remission of the skin lesions and oral cavity, without compromise of other organs.