SMALL-BOWEL FISTULAS IN DEGOS-DISEASE - A CASE-REPORT AND LITERATURE-REVIEW

Degos' disease (malignant atrophic papulosis) is a rare, progressive, small- and medium-size arterial occluding disease, leading to tissue i nfarction and initially involving the skin. We report a case with bowe l involvement followed by enterocutaneous fistulae. Diagnostic laparos copy with jejunal biopsy was followed by jejunal perforations, periton itis, and fistulae leading to death after a 4-month course in the inte nsive care unit. The usual treatment of enterocutaneous fistula by som atostatin and parenteral nutrition was ineffective in this case. The c ourse 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, Dego s' disease should be considered in the differential diagnosis of a pri mary ulceration of the small intestine.