Systemic Langerhans Cell Histiocytosis
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Imaging findings
Chest radiographs and computed tomography show progressive, diffuse interstitial-appearing, lacy, and cystic lung disease with upper-lung predominance and sparing of the lung bases. The patient also has a history of a resected skull lesion, subcutaneous scalp nodules, and pituitary stalk involvement presenting as diabetes insipidus.
Key takeaways
Langerhans cell histiocytosis in young adults is typically smoking-related and isolated to the lungs, presenting with nodular and cystic changes. However, when presenting with systemic findings such as osteolytic bone lesions, cutaneous nodules, and diabetes insipidus, it represents systemic LCH, which is independent of smoking and requires systemic therapy.
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