A 48-year-old man was admitted to our hospital's emergency department with a 2-month history of recurrent abdominal pain and a 2-week history of aggravation. He underwent an exploratory laparotomy for acute diffuse peritonitis 50 days ago at the local hospital. Intraoperative findings revealed that numerous 0.2-0.4 cm yellow-white tubercles were interspersed on the surface of congestive small intestine, colon, appendix, greater omentum and mesenterium, especially the distal ileum (Figure 1a), without apparent perforation. Rapid pathology of appendix and omentum showed acute nonspecific inflammation and intestinal tuberculosis was suspected initially. Therefore large amount of saline and metronidazole was used for irrigation and abdominal drainage was placed. The patient discharged from hospital with intermittent slight abdominal pain on the 15th day after operation.
Twenty days later, the patient developed severe abdominal pain suddenly with nausea and vomiting accompanied by fever (39 C). He was admitted to the local hospital again and transferred to our intensive care unit (ICU) for deterioration of his condition. Physical examination showed evident abdominal tenderness and rebound tenderness with weakened bowel sounds. Meanwhile, generalized skin eruptions with an atrophic porcelain-white center surrounded by erythematous rim were visible over trunk and extremities, measuring 0.2-1.2 cm (Figure 1b). No chronic diseases, no alcohol use, no family history, no herbal agents, or no suspected drug use were reported. Relevant laboratory tests and radiographic results were as follows: WBC 8.65*109 (3.5-9.5*109), NEUT% 0.938  (0.40-0.75), procalcitonin 171.45 (0-0.05 ng/ml), HBV-DNA 9.15E+08  (<5.0E+02 IU/ml). Tuberculin test, Widder test, anti SS-A, and SS-B antibodies were negative. ANA was 1:100 weakly positive and Anti Ro-52 antibodies was positive too. Computed tomography showed a small amount of free air bubble, edema and thickening of the small bowel wall and massive seroperitoneum (Figure 1c). An exploratory laparotomy was performed on account of primary diagnosis of Crohn's disease with intestinal perforations. In the operation, we found more than 100 whitish-yellow plaques, dozens of intestinal perforations and purulent materials (Figure 1d). We resected 2 meters of the perforated small bowel and sutured the seromuscular layer in the wafery areas followed by enterostomy and abdominal drainage tube placement. The diagnosis of Crohn's disease was doubtful for the eccentric appearance of the intestine conditions, but there was still no definite diagnosis.
On the 5th day after operation, intestinal contents drained again, which signified recurrence of perforation. At this point, we eventually started to pay attention to the correlation between the perforation and skin lesions. Biopsy of skin eruption was conducted and the pathology showed atrophy of epidermis, hyperplasia and collagen of dermal fibrous tissue, degeneration of elastic fibers, significantly reduction and partial necrosis of skin appendages, fibrinoid necrosis in small vessels of the deep dermis and thrombosis in local lumen (Figure 1e). Histopathology of the resected intestine showed edema and hyperaemia, thrombus organization of both tiny artery and veins lumen with acute and chronic inflammatory cell infiltration (Figure 1f). Finally, definite diagnosis of Degos disease was concluded. The patient was given aspirin (100 mg/day) and low-dose subcutaneous heparin (5,000 U/dy), but his body condition went from bad to worse because of the severe abdominal inafection. He requested automatic discharge and died 3 days later.