Purpose or Case Report: Malignant atrophic papulosis (MAP), also known as Degos disease, is an exceedingly rare chronic and progressive thrombo-obliterative vasculopathy with only about 200 reported cases worldwide. MAP can present in two forms: (1) a benign skin limited form characterized by pathognomonic skin lesions, or (2) more malignant systemic form that involves thrombo-obliterative vasculopathy of visceral organs; most commonly the gastrointestinal tract, specifically the small bowel. It can also involve the brain, heart, kidneys, or lungs. Visceral MAP is potentially life-threatening and warrants prompt identification and management. Our case is a 17-year-old female presenting to the emergency department with several days of fever, abdominal pain, and vomiting. Additionally, she reported unintentional weight loss and 1 year of generalized rash unresponsive to treatment for presumed Impetigo. Ultrasound revealed moderate ascites. A dermatology consultation including a punch skin biopsy diagnosed MAP for which she was admitted. CT of the abdomen and pelvis revealed diffuse small bowel inflammation, moderate volume complex ascites and small pleural effusion. Brain MRI showed findings concerning for small vessel vasculopathy. Medical treatment for MAP was initiated. Despite initial improvement on medical management and peritoneal drainage, her condition worsened with fever, pain and bilious emesis. Other infectious etiology were investigated and excluded. Surveillance CT persistent small bowel inflammation and dilation in keeping with ileus, pneumatosis intestinalis, and worsening signs of peritonitis without bowel obstruction. Pneumoperitoneum foci were present, at least partially attributable to paracentesis versus perforation. Subsequent bilious drainage from an indwelling peritoneal catheter warranted diagnostic laparoscopy. Small bowel inflammation and yellow serosal lesions characteristic of the MAP ulcers were identified in the operative suite. Additionally, focal ileal perforation was identified and repaired. Her MAP was managed via immunomodulation and she gradually recovered during her lengthy hospital stay. Her brain MRI findings resolved. However, she developed cardiac symptoms, and a cardiac MRI demonstrated reduced LV systolic function without late gadolinium enhancement. She remains hospitalized for management of her cardiac condition. Our case illustrates findings of this rare entity over serial abdominal CT and brain MRI.
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