Pediatric pyoderma gangrenosum: is it just big wounds on little adults?

Soleimani, Tahereh; Sasor, Sarah E.; Spera, Leigh J; Eppley, Barry E.; Socas, Juan; Chu, Michael; Tholpady, Sunil S.

doi:10.1016/j.jss.2016.06.045

Cited by 12 publications

(13 citation statements)

References 23 publications

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“…Our data support the validity of the concept of neutrophilic diseases in a paediatric population and indicate that neutrophilic disease may be more frequent in paediatric patients compared with adults. 16,19 ND occurring in paediatric patients should incite clinicians to schedule complementary explorations in order to search for involvements of other organs.…”

Section: Discussionmentioning

confidence: 99%

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

Bucchia

Barbarot

Reumaux

et al. 2019

Acad Dermatol Venereol

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Background Our suggested ‘modern’ concepts of ‘neutrophilic dermatoses’ (ND) and ‘neutrophilic disease’ were based on observations in adult patients and have not been studied in paediatric patients. Only a minority of ND occurs in children, and little is known about age‐specific characteristics. Objectives To describe age‐specific characteristics of ND in children and to study whether our suggested ‘modern’ classification of ND may be applied to children. Methods We conducted a retrospective multicentre study in a French cohort of 27 paediatric patients diagnosed with pyoderma gangrenosum (PG) or Sweet's syndrome (SS). Results Demographics and distribution of typical/atypical forms were similar in patients diagnosed with PG and SS. Atypical ND were more frequent in infants (90%), when compared to young children (60%) and adolescents (33%). Neutrophilic disease was observed in 17/27 patients and was most frequent in infants. Neutrophilic disease of the upper respiratory tract, as well as cardiac neutrophilic disease, was only observed in infants, whereas other locations were similarly found in infants, young children and adolescents. In infants and young children, ND were associated with a large spectrum of general diseases, whereas in adolescents associations were limited to inflammatory bowel disease and Behçet's disease. Conclusions Our study describes the concept of ND in paediatric patients and shows that they have some characteristics different from ND occurring in adults. ND occurring in infants can be associated with a large spectrum of general diseases. Occurrence of neutrophilic disease is frequent in children. Thus, ND occurring in young paediatric patients should incite clinicians to schedule complementary explorations in order to search for involvement of other organs and to rule out monogenetic autoinflammatory syndromes.

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Section: Discussionmentioning

confidence: 99%

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

Bucchia

Barbarot

Reumaux

et al. 2019

Acad Dermatol Venereol

View full text Add to dashboard Cite

show abstract

“…Skin biopsy demonstrated a neutrophilic infiltrate in the dermis and subcutaneous fat without vasculitis nor granulomas, with negative stains and cultures for pathogenic organisms (Figure and Figure ) consistent with a diagnosis of pyoderma gangrenosum. Pyoderma gangrenosum (PG) is a rare, idiopathic ulcerative skin condition within the spectrum of neutrophilic and autoinflammatory dermatosis that occurs both in adults and children …”

Section: What Is the Diagnosis?mentioning

confidence: 99%

“…Other reported association was pediatric vasculitis with Behcet's and Takayasu's disease or immune deficiency. More recently, PG has been associated with novel drug therapies like pegfilgrastim, gefitinib, and other autoinflammatory syndromes including PAPA (pyogenic arthritis, PG, and acne), PAPASH (pyogenic arthritis, PG, acne, and hidradenitis suppurativa), and PASH (PG, acne, hidradenitis suppurativa) …”

Section: What Is the Diagnosis?mentioning

confidence: 99%

“…Although the effort of some authors to create some diagnostic criteria for PG, they have not been validated . Skin biopsy is important to rule out other causes . Histologic findings are nonspecific and consist of neutrophilic aggregates in the dermis and subcutaneous tissue with some mononuclear cells …”

Section: What Is the Diagnosis?mentioning

confidence: 99%

“…Pyoderma gangrenosum (PG) is a rare, idiopathic ulcerative skin condition within the spectrum of neutrophilic and autoinflammatory dermatosis that occurs both in adults and children. 1,2 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.…”

Section: Diagnosis: Pyoderma Gangrenosummentioning

confidence: 99%

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