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Naim Alkhouri, Vera Hupertz, Lori Mahajan, Adalimumab treatment for peristomal pyoderma gangrenosum associated with Crohn's disease, Inflammatory Bowel Diseases, Volume 15, Issue 6, 1 June 2009, Pages 803–806, https://doi.org/10.1002/ibd.20748
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To the Editor:
Pyoderma gangrenosum (PG) is an uncommon neutrophilic dermatosis first described by Brunsting et al in 1930.1 It is a painful, chronic ulcerating inflammatory skin condition that appears to be immune-mediated. The characteristic lesion begins as small erythematous papules that rapidly spread concentrically, coalesce, and subsequently centrally become ulcerated and necrotic. The mature lesion has a well-defined, undermined, violaceous border and is exquisitely painful. PG develops over the lower extremities in more than 70% of cases; however, any cutaneous area or mucosal surface may be affected.2 More than 50% of PG cases are associated with systemic disease. Patients with inflammatory bowel disease (IBD) account for ≈30% of all PG cases.2 Other associations include rheumatoid arthritis, hematologic malignancies, and intraabdominal malignancies.3 The precise pathogenesis of PG remains unclear. The association of PG with autoimmune disorders and successful management with a variety of local and systemic immunosuppressant agents suggests that disturbances of immune regulation play a role. Immune dysregulation, including defects in neutrophil chemotaxis, neutrophil hyperreactivity, and overexpression of cytokines such as interleukin-8 have been identified. Tumor necrosis factor alpha (TNF-α), a powerful proinflammatory cytokine, may mediate many of these effects and, therefore, play a role in the pathogenesis of PG.2 Anti-TNF-α biotherapies including infliximab, etanercept, and adalimumab offer new therapeutic options for PG resistant to local therapies or other immunosuppressants. Peristomal pyoderma gangrenosum (PPG) is a rare clinical variant of PG that is likely underreported, but has gained recognition by surgeons and medical specialists over the past 2 decades. Similar to PG, the peristomal variant typically affects patients between the ages of 25 and 54 years. There have been only 3 prior reports of this entity in patients 18 years of age or younger.4,–6 Anti-TNF-α therapy with infliximab was effective in 2 of these patients and has also been successful in adults with PPG. We report the novel use of the anti-TNF-α biotherapy agent adalimumab for treatment of PPG in 2 pediatric patients.
