Small bowel fistulae in Degos' disease: a case report and literature review.

Abstract

Degos' disease (malignant atrophic papulosis) is a rare, progressive, small- and medium-size arterial occluding disease, leading to tissue infarction and initially involving the skin. We report a case with bowel involvement followed by enterocutaneous fistulae. Diagnostic laparoscopy with jejunal biopsy was followed by jejunal perforations, peritonitis, and fistulae leading to death after a 4-month course in the intensive care unit. The usual treatment of enterocutaneous fistula by somatostatin and parenteral nutrition was ineffective in this case. The course of the disease in our patient was not usual, as can be seen in a literature review underlining the specific features of Degos' disease. Laparoscopy and bowel biopsy should be avoided in this context. Degos' disease should be considered in the differential diagnosis of a primary ulceration of the small intestine.