Lethal systemic degos disease with prominent cardio-pulmonary involvement.

Abstract:

: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.

journal_name

Indian J Dermatol

authors

Ali YN,Hamed M,Azita N

doi

10.4103/0019-5154.87157

subject

Has Abstract

pub_date

2011-09-01 00:00:00

pages

564-7

issue

5

eissn

0019-5154

issn

1998-3611

pii

IJD-56-564

journal_volume

56

pub_type

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