Treatment of Pyoderma Gangrenosum in Pediatric Inflammatory Bowel Disease

JPGN Reports · 4 authors, 1 centre

AI SUMMARY

FIDELITY 100%

POPULATIONPediatric patients (≤19 years) with inflammatory bowel disease (IBD) and histopathologically confirmed pyoderma gangrenosum (PG)

INTERVENTIONAnti-TNF-α biologic agents (infliximab, adalimumab) as monotherapy or combination therapy

COMPARISONOther treatments including topical/intralesional agents, systemic corticosteroids, calcineurin inhibitors, thiopurines, methotrexate, 5-ASA drugs, granulocyte and monocyte adsorption apheresis, intravenous immunoglobulin, and skin grafting

This summary was generated by AI from a single paper. It has not been reviewed by a clinician and is not clinical advice. Verify against the source before acting on it.

AGENT 01 · STRUCTURED SUMMARY

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TL;DR

This case report and literature review describe a 17-year-old female with ulcerative colitis and severe pyoderma gangrenosum (PG) that was refractory to multiple therapies, including corticosteroids, tacrolimus, dapsone, antibiotics, and skin grafting. Treatment with adalimumab, an anti-TNF-α biologic, led to significant improvement within one month and near-complete healing after six months, with sustained remission for four years. The findings support the use of anti-TNF-α biologics as first-line therapy for severe pediatric IBD-associated PG, especially when conventional treatments fail.

Full summary

3,611 CHARS

**Background:** Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that can occur as an extraintestinal manifestation of inflammatory bowel disease (IBD), particularly ulcerative colitis (UC) and Crohn's disease (CD). In pediatric IBD, PG is associated with significant morbidity and is often refractory to multiple treatments. The pathogenesis involves dysregulation of cytokines, including tumor necrosis factor-alpha (TNF-α), which plays a key role in the inflammatory cascade. This report presents a case of severe PG in a pediatric UC patient and reviews the literature on treatment outcomes in pediatric IBD-associated PG.

**Methods:** The authors describe a 17-year-old female with UC in clinical remission (PUCAI score 0) who presented with a large, painful leg ulcer (15×7 cm) consistent with severe PG. The diagnosis was confirmed histopathologically. Over one year, the lesion was refractory to oral and intralesional corticosteroids, topical and oral tacrolimus, topical dapsone, oral antibiotics (metronidazole, doxycycline), intralesional IL-10, and autologous skin grafting. Subsequently, subcutaneous adalimumab (ADA) monotherapy was initiated using a standard induction and maintenance protocol. For the literature review, the authors searched MedlinePlus and PubMed for English-language articles published between 2002 and 2018 on pediatric IBD-associated PG. Inclusion criteria were: age ≤19 years at diagnosis of histopathologically confirmed PG, PG associated with confirmed IBD, and indication of specific PG treatments and outcomes. Twelve studies met criteria, describing 19 patients (13 females, 6 males; 13 with CD, 6 with UC).

**Key Results:** In the case report, significant improvement in the PG ulcer was observed within one month of starting adalimumab, with almost complete healing after six months. Adalimumab was continued at 40 mg subcutaneously every 2 weeks, and both UC and PG remained in clinical remission after 4 years, with no new PG lesions. In the literature review, 11 patients received systemic corticosteroids for PG, but only 1 achieved complete response with monotherapy. Cyclosporine-A monotherapy was successful in 1 of 4 patients. Thiopurines alone were generally ineffective; 1 patient improved with combination therapy including corticosteroids. Among 9 pediatric patients who received an anti-TNF-α biologic (infliximab or adalimumab), 8 achieved complete PG resolution with monotherapy, including those who had failed other regimens. Topical tacrolimus combined with systemic corticosteroids led to complete response in 3 patients, and 1 patient responded to topical tacrolimus monotherapy. Other therapies (5-ASA, GCAP, IVIG, skin grafting) showed limited or no benefit.

**Clinical Implications:** The findings highlight the efficacy of anti-TNF-α biologics as first-line therapy for severe pediatric IBD-associated PG, particularly in cases refractory to conventional treatments. The case demonstrates that adalimumab can induce and maintain long-term remission of PG even when colitis is in clinical remission. The literature review supports this approach, with 8 of 9 pediatric patients achieving complete PG resolution on anti-TNF-α monotherapy. These results align with adult studies, including a randomized controlled trial showing 69% response to infliximab and a systematic review reporting 92% response rate. The authors recommend anti-TNF-α biologics as first-line therapy for severe PG in pediatric IBD, while acknowledging the need for larger, multicenter randomized controlled trials to establish definitive treatment guidelines.

PICO

PPOPULATION

Pediatric patients (≤19 years) with inflammatory bowel disease (IBD) and histopathologically confirmed pyoderma gangrenosum (PG)

IINTERVENTION

Anti-TNF-α biologic agents (infliximab, adalimumab) as monotherapy or combination therapy

OOUTCOME

Complete clinical response (resolution) of PG lesions

STUDY TYPE

case_report

SPECIALTY

gastroenterology

SUMMARISED BY

AI pipeline

FIDELITY CHECK

100% · A

ORIGINAL PAPER

PMC10191521

Treatment of Pyoderma Gangrenosum in Pediatric Inflammatory Bowel Disease

JPGN Reports

READ THE FULL PAPER

ABSTRACT

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. Keywords: adalimumab, corticosteroids, immunomodulators, infliximab, inflammatory bowel disease, tumor necrosis factor-α

CITATION EXPORT

Katherine Vaidy, Rebecca Winderman, Simon S Rabinowitz et al.. Treatment of Pyoderma Gangrenosum in Pediatric Inflammatory Bowel Disease. JPGN Reports. (2020). https://doi.org/10.1097/PG9.0000000000000008

AUTHORS

4 · 1 CENTRES

Katherine Vaidy, Rebecca Winderman, Simon S Rabinowitz, Steven M Schwarz

1From the Division of Gastroenterology, Hepatology and Nutrition, Children’s Hospital at Downstate, SUNY-Downstate Health Sciences University, Brooklyn, NY.