Langerhans cell histiocytosis with Pulmonary involvement in an infant - A case report

Background:1Langerhans cell histiocytosis (LCH) is characterized by monoclonal proliferation and inltration of organs by Langerhans cell. Pulmonary involvement is common in young adults, but it is uncommon in children. Case summary:A 7 month old female child, presented with shortness of breath and multiple hypopigmented macules with verrucous lesions over scalp. HRCT was chest, showed tiny cysts with cavitatory nodules and nodules with reticulations, likely to represent LCH. Skin biopsy had inltrating epidermis s/o LCH, immunohistochemistry revealed CD1a positive. Hence the diagnosis of Multisystemic Langerhans cell histiocytosis with pulmonary involvement was made. Infant planned to start chemotherapy but deteriorated, and succumbed to the disease. Conclusion:Pulmonary involvement is generally a component of systemic involvement. Therefore, children with persistent respiratory problems should be carefully evaluated for rare entities like Pulmonary LCH.