Treatment of Pyoderma Gangrenosum in Pediatric Inflammatory Bowel Disease

Abstract

Pyoderma gangrenosum (PG) is a rare, necrotizing dermatologic condition associated with neoplastic and immune dysregulatory states, including adult and pediatric inflammatory bowel disease (IBD). Over the last decade, the elucidation of inflammatory mediators in PG has led to a plethora of localized and systemic corticosteroid sparing therapies including antibiotics, antiinflammatory, and immunomodulatory agents. Herein, we describe the case of a 17-year-old female with ulcerative colitis in clinical remission, who presented with a long-standing, large, deep, and painful lower extremity PG lesion. Following failed attempts both at local and at systemic therapies, her PG was successfully treated with the tumor necrosis factor-alpha (TNF-α) monoclonal antibody adalimumab, and the lesion remains in remission after four years of subcutaneous anti-TNF therapy. This case serves as the basis for our presenting a review of the pathogenesis, diagnostic criteria, differential diagnosis, therapies and treatment outcomes for pediatric IBD-associated PG. Our experience adds to earlier reports suggesting anti-TNF-α biologic therapy is most likely to achieve long-term resolution of IBD-associated PG in children and adolescents with severe lesions or who failed other treatments.

Dermatologic manifestations in pediatric patients with inflammatory bowel disease

“…Prevalence of PG was comparable between our subcohorts with CD (2.5%) and UC (2%), although correlation with disease severity was exclusively noted among CD patients. Currently, there is no definite consensus on whether PG is more commonly observed in CD or UC in both children and adults 19–21 …”

“…PG lesions in children with IBD are frequently located in the peristomal areas and lower extremities 19 and seen with both CD and UC. However, a majority of PG patients with IBD are believed to have certain degrees of active colitis 19,20 . IBD‐associated PG tends to have a severe and protracted course.…”

“…Currently, there is no definite consensus on whether PG is more commonly observed in CD or UC in both children and adults. [19][20][21] PG lesions in children with IBD are frequently located in the peristomal areas and lower extremities 19 and seen with both CD and UC. However, a majority of PG patients with IBD are believed to have certain degrees of active colitis.…”

“…However, a majority of PG patients with IBD are believed to have certain degrees of active colitis. 19,20 IBD-associated PG tends to have a severe and protracted course. It is often resistant to first-line therapy with wound care, potent topical steroids, and oral antibiotics (e.g., doxycycline) and often necessitates a more aggressive therapeutic approach, 4 which may involve the use of an anti-TNF, JAK inhibitor, or anti-interleukin (IL) 12/23.…”