An Unusual Case of Gastrointestinal Bleeding That Resolved With Eculizumab Infusion

Abstract

Introduction: Degos disease or malignant atrophic papulosis (MAP) is a very rare autoimmune vasculopathy with only 200 cases reported in the literature by far. The disease is a thrombo-obliterative vasculopathy that primarily involves the skin, gastrointestinal tract, and central nervous system. Here, we report a case of a patient who presented to our hospital with abdominal pain and recurrent episodes of hematochezia. The patient had a colonoscopy, which revealed no detectable cause of bleeding. Subsequent staining of skin and GI biopsies was consistent with the diagnosis of Degos disease. Case Report: A 31-year-old female with a established diagnosis of SLE presented with complaints of diffuse abdominal pain and recurrent episodes of bleeding per rectum. The patient also reported recurrent abrupt outbreaks of papular lesions over her legs and the dorsum of her palms over the past year. Over the span of an hour, these papular lesions would progress to oozing ulcers, eventually forming crater-like lesions, and were associated with severe pain. These lesions were attributed to possible lupus flares, and the patient was placed on a progressively increasing dose of steroids and Cellcept with no relief. Abdominal pain and episodes of bleeding seemed to be associated with these outbreaks, occurring soon after resolution of the lesions or simultaneously. The patient’s vitals were stable on presentation. Abdominal exam was consistent with diffuse abdominal tenderness prominent in the epigastric region with no evidence of guarding, rigidity, or rebound. Several scattered crater-like lesions, 10-15mm in size, with central atrophy and porcelain depression in the lower extremities as well as similar lesions on the dorsum of her palms, were noted. Labs were significant for a hemoglobin of 10g/dL. The patient had an endoscopy that revealed multiple oozing lesions in the gastric antrum and gastric mucosa, with edema and congestion on gastric biopsy. Colonoscopy was significant for mild right-sided colitis. Skin and gastric biopsies were performed and pathology revealed histologic features of Degos disease with positive antiendothelial cell antibody assay. Patient was placed on eculizumab infusion that resulted in rapid resolution of cutaneous and gastrointestinal symptoms. The patient was discharged with planned biweekly infusions. Conclusion: Degos disease is a rare occlusive vasculopathy affecting skin and viscera. The GI tract is involved in >60% of patients with systemic disease. Mortality in these patients is primarily from gastrointestinal complications such as intestinal perforation and sepsis from peritonitis. Early recognition of this disease entity and initiation of therapy with eculizumab is vital and can be life saving.