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  • Title: [Pseudoxanthoma elasticum with pulmonary calcification].
    Author: Yamamoto N, Hasegawa H, Sakamoto H, Numata H, Fukuda T, Komatsu S, Kido Y.
    Journal: Nihon Kyobu Shikkan Gakkai Zasshi; 1996 Jun; 34(6):716-20. PubMed ID: 8741541.
    Abstract:
    A woman born in 1920 had had multifocal, yellowish nodules on her skin since childhood and had been given a diagnosis of pseudoxanthoma elasticum. When she was 59-year-old, a diffuse granular shadow was seen on a chest radiograph. When she was 74-year-old, she presented with dyspnea on exertion. A thoracoscopic lung biopsy was done. Histologic examination of the lung tissue revealed small calcified nodules scattered in the alveolar septa. Pseudoxanthoma elasticum is a systemic, hereditary disorder that usually affects elastic fibers of the skin, eye, and cardiovascular system. It's incidence is quite low: 1 out of 160,000-1,000,000 population. Very few cases of pseudoxanthoma elasticum with pulmonary involvement have been reported, and we know of no previous case in Japan in which pulmonary microcalcification was confirmed histologically.
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