Pediatric pyoderma gangrenosum: a systematic review and update
- 23 February 2017
- journal article
- review article
- Published by Wiley in International Journal of Dermatology
- Vol. 56 (5), 486-495
- https://doi.org/10.1111/ijd.13584
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.Keywords
This publication has 51 references indexed in Scilit:
- Leukocyte adhesion defect type 1 presenting with recurrent pyoderma gangrenosumIndian Journal of Dermatology, 2013
- Idiopathic Infantile Pyoderma Gangrenosum with Stridor Responsive to InfliximabPediatric Dermatology, 2009
- Pyoderma gangrenosum – a reviewOrphanet Journal of Rare Diseases, 2007
- Pyoderma Gangrenosum in an InfantPediatric Dermatology, 2006
- Infantile Crohn Disease Presenting with Diarrhea and Pyoderma GangrenosumPediatric Dermatology, 2006
- Pyoderma gangrenosum of the skin and trachea in a 9-month-old boyJournal of the American Academy of Dermatology, 1996
- Pyoderma gangrenosum: Classification and managementJournal of the American Academy of Dermatology, 1996
- INFANTILE PYODERMA GANGRENOSUMAustralasian Journal of Dermatology, 1992
- Pyoderma gangrenosum in infancy: The youngest reported patientJournal of the American Academy of Dermatology, 1991
- Pyoderma gangrenosum in pediatric acquired immunodeficiency syndromeThe Journal of Pediatrics, 1990