Early-Onset Multisystem Langerhans Cell Histiocytosis With Pulmonary and Cutaneous Involvement in a 3.5-Month-Old Infant: A Case Report Publisher



Khazaei N ; Shirzadi R ; Mirlohi SH
Source: Respiratory Medicine Case Reports Published:2026

Abstract

Background: Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder characterized by the accumulation of Langerhans-type dendritic cells in various tissues, including the lungs and skin [1]. One of the major challenges in this disease is its early diagnosis, the most important reason being that pulmonary involvement is rare in this disease. [2]. Case Presentation: A 3.5-month-old male infant presented to the emergency department of a children's hospital with symptoms of respiratory distress, mild fever, and vesicular-pustular skin lesions. Imaging studies revealed diffuse interstitial lung disease with bronchiectasis and cystic changes. Skin biopsy was positive for immunohistochemical markers CD1a and S-100, confirming the diagnosis of LCH. The patient initially required invasive mechanical ventilation and was subsequently managed with noninvasive mechanical ventilation and systemic corticosteroids, resulting in clinical improvement. Conclusion: Considering the patient presented, attention should be paid to Langerhans Cell Histiocytosis in infants who present with respiratory distress and skin lesions to enable early diagnosis and timely intervention. © 2026 Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license. http://creativecommons.org/licenses/by-nc-nd/4.0/