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. 2011 Sep-Oct;56(5):564-7.
doi: 10.4103/0019-5154.87157.

Lethal systemic degos disease with prominent cardio-pulmonary involvement

Yaghoobi Notash Ali  1 , Mazoochy Hamed, Nikoo Azita
Affiliations

Lethal systemic degos disease with prominent cardio-pulmonary involvement

Yaghoobi Notash Ali et al. Indian J Dermatol. 2011 Sep-Oct.

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.

Keywords: Degos disease; autopsy; clinical manifestation; clinicopathologic correlation; prognosis.

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Conflict of interest statement

Conflict of Interest: Nil.

Figures

Figure 1
Figure 1
Typical skin lesions with atrophic porcelain white center surrounded by erythematous and telangiectatic rim on: (a) trunk, (b) genitalia, (c) palm, (d) sole
Figure 2
Figure 2
T1-weighted Brain MRI from patient showing infarction in right side of pons
Figure 3
Figure 3
Histopathological features of an established lesion (hematoxylin and eosin). (a) Low-power view showing atrophy, ulceration and hyperkeratosis in epidermis, besides a wedge-shape ischemic area with fibrosis in dermis. (b) High-power view showing perivascular lymphocytic infiltration with endothelial swelling
See this image and copyright information in PMC

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