A 28-year-old male was admitted to the hospital with "rash for more than 2 years and abdominal pain for 5 months". The patient began to develop back papules (Figure 1) with central ulcer depression 2 years ago. Skin biopsy performed in another hospital revealed inflammatory infiltration, and no diagnosis and treatment were performed. Five months ago, intermittent abdominal pain, diarrhea, accompanied by fever, no melena, hematemesis and other abnormalities began. One month ago, the symptoms of intestinal perforation appeared after severe abdominal pain. Small intestinal perforation repair and partial omentectomy were performed in an external hospital, and the postoperative recovery was good. Half a month ago, abdominal pain with intestinal perforation appeared again, and laparotomy was performed in the emergency department. During the operation, nearly 100 ulcers were scattered throughout the small intestine (Figure 2), and more than 10 intestinal walls were thin. Perforation repair and intestinal wall biopsy were performed. After operation, anti-infection, anticoagulation, nutritional support and other treatments were given. The patient's condition was complicated. After multidisciplinary consultation, the dermatology department was asked to perform skin lesion biopsy. Combined with the pathological results of intestinal wall biopsy in emergency surgery, the patient was diagnosed as malignant atrophic papulosis involving the intestine, and was given eculizumab, remodulin and other symptomatic treatment. However, the patient's intestinal lesions progressed rapidly, secondary to multiple intestinal perforations and intestinal necrosis, and died after active rescue measures such as puncture and surgical drainage failed.