Abstract
BACKGROUND: Pyoderma gangrenosum (PG) is a rare cause of skin ulcers in children, posing challenges in diagnosis and treatment. As the disease is often associated with conditions such as inflammatory bowel disease (IBD), rheumatoid arthritis, haematological disorders and other diseases, diagnosis and treatment often require cooperation with other medical departments. Accordingly, dissemination of information about the disease to doctors in departments other than dermatologists, especially paediatricians, can help in its early detection. CASE PRESENTATION: The 11-year-old pediatric patient in the case initially diagnosed with acute febrile neutrophilic dermatosis was eventually confirmed as pustular PG through histopathological examinations of skin and other relevant examinations. The medical condition is lessened after treatment with a combination of glucocorticoids and adalimumab. CONCLUSION: PG is relatively rare in clinical settings, particularly among pediatric patients exhibiting persistent high fever and signs of pustular pyoderma gangrenosum. This case underscores the importance of considering the potential diagnosis of pediatric pustular PG when confronted with a child presenting persistent high fever and pustules after trauma. Additionally, the proactive initiation of adalimumab emerges as a promising treatment option for pediatric IBD -associated pustular PG.