title: Pediatric pyoderma gangrenosum: a systematic review and update.
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.
authors.affiliation: Department of Dermatology, Hotel Dieu de France University Hospital, Beirut, Lebanon.|Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.
authors.forename: Elio
authors.initials: E
authors.lastname: Kechichian
authors.affiliation: Department of Dermatology, Hotel Dieu de France University Hospital, Beirut, Lebanon.|Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.
authors.forename: Roger
authors.initials: R
authors.lastname: Haber
authors.affiliation: Department of Dermatology, Hotel Dieu de France University Hospital, Beirut, Lebanon.|Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.
authors.forename: Nadim
authors.initials: N
authors.lastname: Mourad
authors.affiliation: Department of Dermatology, Hotel Dieu de France University Hospital, Beirut, Lebanon.|Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.
authors.forename: Rana
authors.initials: R
authors.lastname: El Khoury
authors.affiliation: Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.|Department of Plastic and Reconstructive Surgery, Hotel Dieu de France University Hospital, Beirut, Lebanon.
authors.forename: Samer
authors.initials: S
authors.lastname: Jabbour
authors.affiliation: Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.|Chief of Department of Dermatology, Hotel Dieu de France University Hospital, Beirut, Lebanon.
authors.forename: Roland
authors.initials: R
authors.lastname: Tomb
mesh_terms: D000152:Acne Vulgaris / Q000150:complications / Q000188:drug therapy; D000293:Adolescent; D001170:Arthritis, Infectious / Q000150:complications / Q000188:drug therapy; D002648:Child; D002675:Child, Preschool; D006402:Hematologic Diseases / Q000150:complications / Q000188:drug therapy; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000188:drug therapy; D007223:Infant; D007231:Infant, Newborn; D015212:Inflammatory Bowel Diseases / Q000150:complications / Q000188:drug therapy; D017511:Pyoderma Gangrenosum / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D014657:Vasculitis / Q000150:complications / Q000188:drug therapy