Bowel-associated dermatosis-arthritis syndrome in a child with very early onset inflammatory bowel disease

Area of Science:

• Pediatric Gastroenterology
• Dermatology
• Immunology

Background:

• Inflammatory bowel disease (IBD) encompasses chronic gastrointestinal inflammation.
• Very early-onset IBD (VEO-IBD) presents before age 6 and often has severe manifestations.
• Bowel-associated dermatosis-arthritis syndrome (BADAS) is a rare extraintestinal manifestation linked to IBD.

Observation:

• A 6-year-old boy with VEO-IBD experienced high fevers, diarrhea, arthralgias, and myalgias.
• He developed a progressive papular and vesiculopustular rash on his face, trunk, and extremities.
• Histopathology revealed dense neutrophilic inflammation in the dermis.

Findings:

• The clinical presentation and histopathological findings were consistent with BADAS.
• This syndrome is infrequently reported in pediatric populations.
• The case underscores the diverse dermatological manifestations of VEO-IBD.

Implications:

• This case expands the understanding of BADAS in children.
• Early recognition and diagnosis of BADAS are crucial for appropriate management in pediatric IBD.
• Further research is warranted to elucidate the pathogenesis and optimize treatment strategies for BADAS in pediatric patients.