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Cutaneous polyarteritis nodosa in a child with positive antiphospholipid and P-ANCA
B A Pereira1, N A Silva, A C Ximenes
1Department of Internal Medicine/Rheumatology Unit, Hospital das Clínicas, Faculty of Medicine, Federal University of Goiás (HC-FMUFG), Brazil.
Insights
Juvenile cutaneous polyarteritis nodosa (PAN) can cause severe skin issues and gangrene. This case highlights a rare co-existence of cutaneous PAN with antiphospholipid antibodies and perinuclear antineutrophil cytoplasmic antibodies.
Area of Science:
- Pediatric Rheumatology
- Dermatology
- Immunology
Background:
- Juvenile cutaneous polyarteritis nodosa (PAN) is a rare vasculitis affecting children.
- Diagnosis and treatment remain challenging due to varied presentations.
- Limited data exists on specific antibody associations in pediatric cutaneous PAN.
Observation:
- A case study of a child with recurrent fever, rash, and digital gangrene since infancy.
- Clinical manifestations were primarily limited to the skin and musculoskeletal system.
- The patient experienced episodes of hypertension and persistent eosinophilia.
Findings:
- The presented case uniquely exhibits cutaneous PAN alongside antiphospholipid antibodies (aPL) and perinuclear antineutrophil cytoplasmic antibodies (p-ANCA).
- This co-occurrence has not been previously reported in the literature.
- The findings suggest potential novel pathogenic mechanisms or diagnostic markers.
Implications:
- This case expands the understanding of potential autoimmune markers in juvenile cutaneous PAN.
- Further research is warranted to explore the significance of aPL and p-ANCA in this condition.
- This may lead to refined diagnostic criteria and targeted therapeutic strategies for pediatric vasculitis.
Abstract:
A case of juvenile cutaneous polyarteritis nodosa (cutaneous PAN) is presented. Since early infancy the child underwent attacks of fever and cutaneous rash that occasionally progressed to gangrene and amputations of distal portions of toes and fingers. Although occasional episodes of high blood pressure and persistence of moderate eosinophilia were present, the clinical pattern was mostly restricted to the musculoskeletal system and skin. The authors discuss the definition of the disease and its present therapeutic possibilities, calling attention to a feature not referred in previous reports: the unique co-existence of cutaneous PAN plus antiphospholipid antibodies (aPL) and perinuclear antineutrophil cytoplasmic antibodies (p-ANCA).