Langerhans cell histiocytosis (LCH) presenting as pneumothorax and neck lymphadenopathy

Abstract

Masoud Mohamadpour, Bahareh Yaghmaii, Seyed Abbas Hassani, Meisam Sharifzadeh, Zeinab Najafi, Anahita Majmaa and leila Tahernia

Langerhans Cell Histiocytosis (LCH) is a rare clonal disease characterized by dendritic cells infiltration in various main organs. We report a 6-month-old infant with severe respiratory distress, cervical lymphadenopathy, and reddish skin rashes on face. Diagnostic work up revealed spontaneous pneumothorax on Chest X-ray (CXR). Multiple thin-walled lung cysts and emphysema was discovered on spiral chest CT scan. Lung tissue and cervical lymph node biopsy revealed dendritic cells infiltration. Broncho alveolar lavage (BAL), cervical lymph node and lung tissue biopsy was performed and histopathology was assessed. It was suggestive of LCH with positive immunohistochemistry markers including CD1a, CD68 and S100.

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