Skin gangrene as an extraintestinal manifestation of inflammatory bowel disease

Abstract

Inflammatory bowel diseases can commonly present many cutaneous lesions which can contribute to the diagnosis of the disease or its activity. The most frequent cutaneous or mucocutaneous manifestations suggesting ulcerative rectocolitis activity are erythema nodosum (3-10%), pyoderma gangrenosum (5-12%) and aphthous stomatitis (4%). Other reactive skin manifestations related to immunological mechanisms associated with the inflammatory bowel disease are: Sweet's syndrome, arthritis-dermatitis syndrome associated with inflammatory bowel disease and leukocytoclastic vasculitis. We describe the case of a young man with diagnosis of ulcerative rectocolitis, which presented an extensive cutaneous gangrene secondary to microvascular thrombosis. The case represents a dermatologic rarity and should be recognized as a cutaneous manifestation related to the hypercoagulability state observed in the disease's activity.

Cutaneous thrombotic vasculopathy related to poorly controlled ulcerative colitis

“…Cutaneous gangrene resulting from thrombotic vasculopathy is a rare and poorly understood extraintestinal manifestation of inflammatory bowel disease, most commonly ulcerative colitis. 1 , 2 , 3 , 4 , 5 Hypercoagulable states and thromboembolism are more common in patients with inflammatory bowel disease compared to control subjects, possibly because of thrombocytosis, abnormal platelet function, endothelial dysfunction, elevation of serum procoagulants (ie, fibrinogen, factor V, factor VII, and von Willebrand factor), and depletion of anticoagulants (ie, antithrombin, protein C, and protein S). 6 , 7 Depletion of antithrombin may be linked to losses through a damaged enteric barrier, because elevated antithrombin levels have been detected in the stool of patients with ulcerative colitis and acquired antithrombin deficiency.…”

“…Cutaneous gangrene caused by thrombotic vasculopathy is a rarely described extr-intestinal complication of ulcerative colitis. 1 , 2 , 3 , 4 , 5 We report a case of cutaneous thrombotic vasculopathy associated with poorly controlled ulcerative colitis, possibly caused by enteric losses of antithrombin III.…”

Thrombotic cutaneous gangrene associated with ulcerative colitis

“…Other manifestations include erythema nodosum, erythema multiforme, perineal fistulas, cutaneous dermatoses, and less frequently, thrombotic cutaneous gangrene. 1 , 2 Thrombotic cutaneous gangrene is a particularly rare complication but has been previously associated. 1 , 2 , 3 , 4 , 5 As the name suggests, focal microvascular thrombosis is thought to be the etiology, secondary to a procoagulant state in this condition.…”

“… 1 , 2 Thrombotic cutaneous gangrene is a particularly rare complication but has been previously associated. 1 , 2 , 3 , 4 , 5 As the name suggests, focal microvascular thrombosis is thought to be the etiology, secondary to a procoagulant state in this condition. On histology, microthrombi can be seen in the superficial dermis.…”