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Pediatric pyoderma gangrenosum: a systematic review and update
Elio Kechichian1,2, Roger Haber1,2, Nadim Mourad1,2
1Department of Dermatology, Hotel Dieu de France University Hospital, Beirut, Lebanon.
Insights
Pediatric pyoderma gangrenosum (PG) is rare, with inflammatory bowel disease being a common associated condition. Treatments vary, but high cure rates of 90% are achievable with tailored approaches.
Area of Science:
- Pediatric Dermatology
- Immunodermatology
- Rheumatology
Background:
- Pyoderma gangrenosum (PG) is a rare neutrophilic disorder in children.
- Limited data exists on pediatric PG's clinical, epidemiological, and therapeutic aspects.
- Numerous associated diseases and treatments necessitate updated reviews.
Purpose of the Study:
- To systematically review recent data on pediatric pyoderma gangrenosum.
- To consolidate information on associated conditions, clinical presentations, and treatments.
- To provide an updated overview for managing pediatric PG.
Main Methods:
- Systematic literature review conducted.
- Searched Embase, Medline, and Cochrane databases.
- Included 132 relevant articles.
Main Results:
- Inflammatory bowel disease, hematologic disorders, vasculitis, immune deficiencies, and PAPA syndrome are common associated diseases.
- Over half of cases have no identified underlying disease.
- Multiple disseminated ulcers are the most frequent presentation.
- Treatments include systemic steroids, dapsone, cyclosporine, and TNF-alpha inhibitors.
- High response rates with cure rates up to 90% were observed.
Conclusions:
- Pediatric PG management requires a high index of suspicion and thorough workup.
- Treatment must be individualized based on the underlying cause.
- Recent data highlights diverse etiologies and effective therapeutic options.
Abstract:
Pyoderma gangrenosum (PG) is a sterile neutrophilic disorder that rarely affects children. Clinical, epidemiological, and therapeutic data on pediatric PG is poor as there are many newly reported associated diseases and drugs. This paper aims to review all recent available data on pediatric PG. A systematic review of the literature was conducted using Embase, Medline, and Cochrane databases. A total of 132 articles were included in the review. The most commonly reported underlying diseases in pediatric PG are inflammatory bowel diseases followed by hematologic disorders, vasculitis, immune deficiencies and Pyogenic Arthritis, Pyoderma gangrenosum and Acne (PAPA) syndrome. More than half of the cases occur with no underlying disease. The most frequently reported clinical presentation is multiple disseminated ulcers. Treatment should be tailored according to the underlying etiology. It includes systemic steroids, corticosteroid sparing agents such as dapsone and cyclosporine, and TNF-alpha inhibitors such as adalimumab and infliximab. Response to treatment is high with cure rates reaching 90%. A high index of suspicion and a thorough workup are mandatory in the management of pediatric PG.
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