Medical University of South Carolina Charleston, SC
Introduction: Acute febrile neutrophilic dermatosis (Sweet’s syndrome) is a rare extraintestinal manifestation (EIM) of inflammatory bowel disease (IBD). Other neutrophilic dermatoses include pyoderma gangrenosum and erythema nodosum. Only 40 cases associated with Crohn’s disease have been reported in the literature. Characteristics include abrupt onset of painful erythematous nodules with fever, arthralgias, and leukocytosis which typically resolve with systemic corticosteroids.
Case Description/Methods: We describe a case involving a 22-year-old male with presumed UC associated fulminant colitis and perforation requiring ostomy. During staged proctocolectomy with plans for a J pouch, he underwent flexible sigmoidoscopy with biopsies consistent with UC. He was started on high dose Infliximab. One month after being placed in continuity as the final stage of J pouch creation, he developed fevers and wound dehiscence. Imaging revealed fistula formation giving rise to the concern for Crohn’s colitis. Despite antibiotics, his fevers persisted.
During this hospitalization, he developed red papules and pustules with peripheral scaling on his face spreading across his body (picture 1) with evidence of pathergy at IV sites. Dermatology diagnosed him with acute febrile neutrophilic dermatosis. Despite the concern for poor wound healing, he was treated with IV corticosteroids 1mg/kg for 5 days with resolution of his lesions. He remains stable on Infliximab.
Discussion: Most commonly, Sweet's Syndrome is reported in women (87%) and associated with active disease (67-80%). It is more common in Crohn's disease than in UC. Early recognition is important in order to start corticosteroids quickly in concert with traditional IBD therapy.
Not only does this case demonstrate a rare finding of Sweet’s syndrome in a male with Crohn’s, it brings up an important discussion about acute systemic corticosteroid therapy initiation during the perioperative period. It is known that corticosteroids affect the process of wound healing. This case highlights a favorable outcome despite use of high dose steroids in this patient who was critically ill requiring treatment. In review of existing literature, 1 randomized double-blind study evaluated the effect of acute administration of steroids in the perioperative period. This study discovered no wound infections and only 1 patient with wound dehiscence. Sweets is a rare EIM and in severe presentations, systemic steroids are indicated, despite presenting in the immediate perioperative period.
