Thrombotic Cutaneous Gangrene: A Rare Dermatologic and Hematologic Extraintestinal Manifestation of Ulcerative Colitis

Abstract

Ulcerative colitis (UC) is associated with many extraintestinal manifestations, which can include dermatologic and hematologic involvement. Thrombotic cutaneous gangrene (TCG) is a rare dermatologic and hematologic complication with potentially poor prognosis, often needing colectomy in addition to anti-coagulation for complete resolution. A 33-year-old healthy female presented with chronic bloody diarrhea and was diagnosed with left sided UC. She was initiated on steroids and discharged on a prednisone taper. Three days after discharge, she presented with fever and progressive and extensive racemose and retiform purpura on her trunk and extremities. She continued to have bloody stool despite adherence to prednisone. Areas of purpura evolved to tense bullae which erupted. She had an elevated D-dimer, prolonged PT, and reduced anti-thrombin III activity. Complete antiphospholipid syndrome panel was negative. A skin punch biopsy showed thrombotic vasculopathy involving small dermal vasculature without vasculitis. No other end-organ complications of hypercoagulability were identified. MRE showed inflammatory changes limited to the descending colon, sigmoid colon, and rectum, with no involvement of the small bowel. Anti-thrombin III levels were replenished, high dose heparin drip was started, and her hypercoagulable state resolved. The patient's UC was refractory to steroids; she was subsequently started on Infliximab, which controlled her luminal symptoms. She was discharged on Lovenox, which significantly improved her skin manifestations. Our case underlines the importance of appreciating a hypercoagulable state in UC and suspecting TCG in UC cases presenting with rapidly progressing purpura. Immediate hypercoagulability work up, skin biopsy, and anticoagulation should be initiated to prevent further complications. Furthermore, we demonstrate that trial of biologic immunosuppressive therapy may prevent need for colectomy to treat TCG.2098_A Figure 1. Retiform purpura on shoulder and back2098_B Figure 2. Areas of purpura evolved to become tense bullae which eventually erupted.2098_C Figure 3. Mayo 3 UC