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.

Age‐specific characteristics of neutrophilic dermatoses and neutrophilic diseases in children

“…In contrast, in adolescent's IBD was the largely predominant associated disease. In agreement with our observations in adolescents, IBD represents the most common general disease associated with ND, accounting for 20–30% of adult PG cases . Thus, in young children presenting with ND, clinicians need to consider the possibility of an association with a larger spectrum of general diseases and genetic autoinflammatory syndromes.…”

“…The extrapolation of these data to the French population (approximately 65 million) is not evident as environmental factors may account for a high variability of the incidence, as suggested by a study in Switzerland . Nevertheless, even when considering that paediatric cases account only for approximatively 4–5% of all PG and SS cases it is likely that our study is far from being exhaustive . Furthermore, recruitment of patients via calls to medical societies may have biased the selection in favour of severe, atypical and/or extracutaneous forms of ND.…”

“…The size of our study cohort may appear relatively small compared with adult cohorts. However, it seems important to note that current data on paediatric ND are limited to single case reports and small case series . This can be illustrated by a recent systematic review of the literature on paediatric PG which included 170 patients who had been reported in 132 publication .…”

“…The development of the concepts of ND and neutrophilic diseases was based on observations in adults, probably because of the rarity of ND in children . Indeed, published data on ND in children are generally limited to reports on either PG or SS . Thus, the validity of our suggested ‘modern’ classifications systems of ND has not been studied in paediatric patients.…”

Cutaneous ulceration in an infant

“…PG cases in infants and adolescents are rare and it accounts for only 4% of PG, with an average age of 9.5 years. Diagnosis of pediatric PG is often delayed, with a median time from initial symptom to diagnosis of 2 months . Clinical appearance is similar in adults and children, although in contrast to adults, PG lesions tend to be disseminated and multiple in the pediatric population.…”