Correct!
3. Langerhans cell histiocytosis
All the diagnosis has been associated with pneumothoracies (1). LAM is a rare disease that is associated with cystic lung disease, angiomyolipomas, and obstruction of the lymphatic system. Birt-Hogg-Dubé syndrome (BHD) is a hereditary condition associated with multiple non-cancerous (benign) skin tumors, lung cysts, and an increased risk of kidney lesions (cysts, benign tumors, and kidney cancer). Lymphoid interstitial pneumonia (LIP) is an uncommon form of interstitial lung disease in adults that is characterized histopathologically by infiltration of the interstitium and alveolar spaces of the lung by lymphocytes, plasma cells, and other lymphoreticular elements.
Langerhans cell histiocytosis (LCH) is the patient's diagnosis as you may have suspected from the title. The disease has gone by several other names, including eosinophilic granuloma (EG, hence the Egg in the title), Hand–Schüller–Christian disease, Abt-Letterer-Siwe disease, and histiocytosis X. LCH is a rare disease involving clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes (2). Clinically, its manifestations range from isolated bone lesions to multisystem disease. Chest radiographs in patients with LCH reveal diffuse reticulonodular infiltrates that are symmetric with sparing of the lung bases a frequent finding. Unlike other interstitial pneumonitides, the lung volumes may be normal or increased and often present with a mixed obstructive and restrictive picture on pulmonary function testing.
Our patient’s diagnosis was confirmed when his pathology from his bone lesion returned. His alpha-1 antitrypsin level was normal.
Which of the following is the recommended initial treatment in patients with LCH? (Click on the correct answer to be directed to the fifth and final page)