[A case of cytokeratin 19 positive extramammary Paget's disease with pulmonary metastases].

: An 82-year-old woman was admitted because of progressing cough and dyspnea. Two years prior to admission, she had undergone an operation for extramammary Paget's disease which resected the primary lesion, the left labium majora, and ipsilateral inguinal and obturator lymph nodes. Thoracic images showed diffuse infiltrative shadows and bilateral effusions. Repeated thoracenteses revealed no microbes or malignant cells. Antibiotic treatment was ineffective. Transbronchial lung biopsy specimens revealed Paget cells, and immunohistochemical stains confirmed pulmonary metastases of Paget's disease. Cytokeratin 19 fragments were positive in the serum, pleural effusion, and lung biopsy specimens. The patient died of respiratory failure 3 years after the initial diagnosis of extramammary Paget's disease.