Indian Journal of Dermatology
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Year : 2011  |  Volume : 56  |  Issue : 5  |  Page : 564-567

Lethal systemic degos disease with prominent cardio-pulmonary involvement

1 Department of Surgery, Sina Hospital, Tehran, Iran
2 Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
3 Department of Pathology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran

Correspondence Address:
Mazoochy Hamed
Department of Dermatology, Tehran University of Medical Sciences, Razi Skin Hospital, Tehran
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.87157

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A 48-year-old man presented with acute abdominal pain underwent laparotomy that revealed two perforated ulcers in jejunum. He had skin lesions with porcelain white atrophic scar which were ignored for 3 years, whereas the disease revealed own malignant nature and progressed to nervous, gastrointestinal, and cardiopulmonary systems. The diagnosis of Degos disease was established on the basis of clinical and histopathological features. He expired due to cardio-pulmonary insufficiency after 5 months from the onset of systemic involvement. Autopsy showed diffuse fibrotic changes in serosal membranes and internal organs. Distribution of skin lesions that involved palmoplantar surfaces, genitalia and scalp and, furthermore, course of disease as rapid progressive cardio-polmunary involvement are remarkable point in this patient. On the other hand, this case highlights importance of clinicopathologic correlation, specially in the dermatologic field.

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