Severe Diffuse Takayasu Arteritis Manifestation in Childhood
María Estela Cano de Santayana Domínguez1, Safeer Jamil2, Debora Luciano1
1Paediatric Cardiothoracic Department, Alder Hey Children's Hospital, Liverpool, United Kingdom.
JACC. Case Reports
|August 29, 2025
Summary
This case report details a rare instance of childhood Takayasu arteritis (TA) co-occurring with pyoderma gangrenosum (PG). Despite aggressive treatment for PG, the child developed severe, widespread vasculitis, underscoring the complexity of these conditions.
Area of Science:
- Pediatric Rheumatology
- Vascular Inflammation
- Autoimmune Diseases
Background:
- Takayasu arteritis (TA) is a rare large-vessel vasculitis affecting the aorta and its branches.
- Pyoderma gangrenosum (PG) is an inflammatory ulcerative skin condition.
- The co-occurrence of TA and PG in children is exceptionally rare.
Observation:
- A 4-year-old boy with a history of pyoderma gangrenosum (PG) presented with neurological symptoms.
- Computed tomography angiography revealed acute inflammatory vasculitis, leading to a diagnosis of Takayasu arteritis (TA).
- Vascular imaging demonstrated extensive TA affecting the aorta and pulmonary artery.
Findings:
- The patient exhibited severe, diffuse vasculitis despite prior immunosuppressive therapy for PG.
- This case represents one of only 10 reported instances of childhood TA and PG co-occurrence.
- Treatment involved a switch to corticosteroids, cyclophosphamide, and antiplatelet therapy.
Implications:
- Highlights the diagnostic challenges and potential for severe systemic involvement in co-existing TA and PG.
- Emphasizes the need for multidisciplinary surveillance in pediatric vasculitis cases.
- Contributes to the limited literature on the combined management of these rare conditions in children.
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