Fatal Presentation of Thrombotic Cutaneous Gangrene Associated With Acute Severe Colitis
AIBD 2023
Background:
Acute severe colitis (ASC) is a life-threatening complication of ulcerative colitis (UC) that requires urgent intervention. It is the initial presentation in 15% of patients with 10-20% risk of colectomy and 1% mortality risk during hospitalization. ASC can manifest with complex coagulalopathies with increased risk of thrombosis and bleeding. Thrombotic cutaneous gangrene is a rare complication and has been previously reported in the literature. The pathophysiology is related to focal microvascular thrombosis secondary to a procoagulant state.
Methods:
After excluding thrombophilia, the management consists of combinations of systemic steroids and anticoagulants.
Case Report:
A 36-year-old female with ulcerative colitis presented to a quaternary hospital with severe abdominal pain and bloody diarrhea. She reported 40-pounds weight loss and low grade fever for the past six months before admission. On physical examination, she was pale and dehydrated, despite hemodynamic stability. Abdominal examination showed distended abdomen and diffuse pain to palpation. Past medical history included ulcerative colitis diagnosed two years ago and diabetes mellitus. She had been treated with mesalamine and oral hypoglycemic drugs. She was a non-smoker and denied alcohol abuse. Initial laboratory workup included hemoglobin of 6.2 g/dL, CRP of 192 (normal range < 5mg/L) and low serum albumin of 2.1 g/dL. CT scan showed diffuse parietal colonic thickening with mucosal hyperenhancement associated with enlarged mesenteric lymph nodes. Flexible sigmoidoscopy showed deep ulcerations and colonic biopsies were negative for CMV and dysplasia. She was treated with hydration, electrolyte replacement and methylprednisolone (0.8 mg/kg/day).
Results:
After three days, rescue therapy with infliximab (5 mg/kg) was initiated due to partial response to corticosteroids. Despite thromboprophylaxis with low-molecular-weight-heparin, the patient developed severe pain and cyanosis of the lower limbs with fixed necrosis of extremities. CT angiography of the lower limbs and transthoracic echocardiography were negative for peripheral thrombosis and pulmonary thromboembolism. An extensive investigation excluded primary causes of thrombophilia. She was given therapeutic anticoagulation, but developed massive lower gastrointestinal bleeding and required subtotal colectomy with protective ileostomy. On the third postoperative day, she was diagnosed with acute thrombosis of the right common iliac veins and anticoagulation with low-molecular-weight-heparin was resumed. She developed a second episode of bleeding with a drop in hemoglobin from 9.0 g/dL to 5.0 g/dL due to retroperitoneal hematoma diagnosed on CT scan. The patient required two reoperations for intraperitoneal hemorrhage. While on intensive care, she developed multiple infectious complications and died of multiorgan failure.
Conclusions:
Ulcerative colitis is associated with a hypercoagulable state and occlusion of small blood vessels causing localized gangrenous changes is a rare complication of the disease. Procoagulant factors are increased during active inflammation, whereas natural anticoagulants and fibrinolytic activity are decreased. In this scenario it is paramount to ensure monitoring of procoagulant disorders and intrinsic risk of bleeding to avoid severe complications.
