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Pediatric pyoderma gangrenosum: a systematic review and update.

Elio Kechichian^1,2, Roger Haber^1,2, Nadim Mourad^1,2

¹Department of Dermatology, Hotel Dieu de France University Hospital, Beirut, Lebanon.

International Journal of Dermatology

|February 25, 2017

Summary

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.

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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.