Langerhans cell histiocytosis: a rare cause of exudative enteropathy in a young child

Exudative enteropathy is a clinical syndrome characterized by excessive loss of proteins through the gastrointestinal tract and rare complication various diseases. One rarest causes protein malabsorption Langerhans cell histiocytosis, which occurs as result inflammatory neoplasia myeloid precursor cells caused mutations in mitogenactivated kinase pathway. Abnormal proliferation accumulation intestinal wall leads to violation outflow lymph, manifestations characteristic enteropathy. Given that lesion with histiocytosis from 2–3% cases, signs are not highly specific, timely diagnosis difficult. Delayed verification late initiation adequate treatment risk factors for multisystem lesions lead an unfavorable outcome. The literature describes few observations onset or manifestation disease symptoms malabsorption. We present case diagnosing young child severe exudative at onset. patient's parents gave their consent use child's data, including photographs, research purposes publications.Refractory require mandatory endoscopic histological examination identify Timely targeted therapy vemurafenib combination subsequent chemotherapy provided favorable prognosis stable remission disease.