Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease. Young male adults develop the disease most frequently. Greater than 90% of cases of PLCH occur in smokers. Diabetes insipidus (DI) occurs in about 11% to 40% of patients and pneumothoraces occur in 10% to 30% of patients. The diagnosis of PLCH can be confirmed by BAL, transbronchial biopsy, or surgical lung biopsy. CASE REPORT A 19-year-old male presented with a four-week history of dyspnea and hemoptysis which

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... ad begun after the appendectomy operation, which was performed in emergency conditions, and worsened with time. Previous medical details were not remarkable except for a history of frequent thirst and excessive urine volume. He had also a smoking history of 1 pack/ year. Physical examination revealed that the patient was tachypneic, but not in respiratory distress, with a respiratory rate of 32 breaths per min. He had no cyanosis and edema. There were erythematous papular skin lesions at axilla, postauricula and scalp. On auscultation rales were present bilaterally at the mid and lower zones of the lungs. Chest radiography revealed diffuse micronodular and interstitial-appearing infiltrates and small cystic air spaces (Fig. 1) . High resolution computed tomography (HRCT) scan showed bilaterally uniform thin-walled cysts of 1-1.5 cm in diameter suggesting PLCH and Lymphangiomyomatosis (Fig. 2) . Pulmoner Langerhans hücreli histiyositoz (PLCH) nadir görülen bir interstisyel akciğer hastalığıdır. Tahmini prevalansı milyonda iki ila beş arasında olup özellikle genç erkeklerde görülür. Olguların %90'ından fazlası sigara içenlerde görülür. %10-%40'ında diabetes insipidus eşlik eder ve %10-%30'unda pnömotoraks görülebilir. Tanı BAL, transbronşiyal biyopsi veya açık akciğer biyopsisi ile konur. Apandektomi operasyonu sonrası gelişen dispne şikayeti ile başvuran, diabetes insipidusun eşlik ettiği ve transbronşiyal akciğer biyopsisi ile PLCH tanısı alan olguyu sunuyoruz. Anahtar sözcükler: Pulmoner Langerhans hücreli histiyositoz; intertisyel akciğer hastalığı; diabetes insipidus; transbronşiyal akciğer biyopisi; dispne. Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease. Its estimated prevalence is two to five cases per million. Young male adults develop the disease most frequently. Greater than 90% of cases of PLCH occur in smokers. Diabetes insipidus (DI) occurs in about 11% to 40% of patients and pneumothoraces occur in 10% to 30% of patients. The diagnosis of PLCH can be confirmed by BAL, transbronchial biopsy, or surgical lung biopsy. We present a case referred to us with dispnea developed after appendectomy operation, coexisting with DI, and diagnosed to have PLCH using transbronchial lung biopsy.