Introduction: Degos disease (DD), also known as malignant atrophic papulosis, is a rare arteriopathy involving the occlusion of small vessels, resulting in ischemia of the supplied tissues. The cause of DD is unknown, but it initially manifests as skin lesions and may progress to other organ systems, predominantly the gastrointestinal tract. It is often fatal, mostly due to small bowel perforations and subsequent sepsis. Here we describe the critical care management of a Degos disease patient with stomach and small bowel perforations.
Description: A 58-year-old Caucasian male with a history of vocal cord paralysis, an intraabdominal abscess, and a penile ulcer presented to the emergency department with abdominal pain, nausea, weight loss, and skin lesions of a nine-month course. Examination revealed a hoarse voice, pale lips, and small, non-pruritic, violaceous macules over the torso and extremities. His white count was 9.5 x 103 / uL, and metabolic abnormalities were present. An abdominal CT scan revealed pneumatosis with a clinically benign abdomen. Sepsis was determined, and he was admitted to intensive care and started on broad-spectrum antibiotics. After admission, the patient developed a right foot drop and hypoxic respiratory failure due to aspiration. Multiple services were consulted, and an underlying vasculitis was suspected. Many skin lesions were biopsied, and an exploratory laparotomy was performed after the development of an acute abdomen. Operative findings revealed extensive necrosis and perforations in the distal jejunum and stomach. Biopsy, small bowel resection, anastomosis, and patching of the stomach wall were performed. Post-operation, the pathology report confirmed Degos disease, leading to the initiation of Eculizumab treatment. The patient clinically improved and was ultimately discharged to a rehabilitation hospital after 28 days post-op.
Discussion: Degos Disease is a complex disorder that is often difficult to treat. The small intestine is the most common perforation site, but this case demonstrated stomach perforation as well. Currently, there is no standard treatment for DD, but Eculizumab shows promise in the literature. In addition, laparotomy was vital in the timely diagnosis and management of our patient’s critical condition.
