Congenital Langerhans cell histiocytosis: A case report

Langerhans cell histiocytosis (LCH) is a rare pathology, with different clinical manifestations in the neonatal period, ranging from isolated bone lesions to a systemic compromise. A case of Langerhans cell histiocytosis is reported with literature review focusing on clinical manifestations, diagnosis and treatment. One month old patient consulted for presenting lymphadenopathy and skin lesions, which was initially managed as an infectious pathology. The disease continued its progression and without improvement with the treatment the patient dies due to respiratory failure. The lymph node and skin biopsy revealed infiltration of atypical cells with immunohistochemistry positive for S100, CD1 and CD68, confirming the LCH. This alteration is a rare disease of low incidence with a variety of clinical manifestations. representing a great challenge. Therefore, it is important to alert and sensitize the medical team about this disease for a diagnosis and timely management.