- 2023, Volume 54 - Number 3
- Published: August 7, 2023
Acute limb ischemia as presentation of pediatric antiphospholipid syndrome: case report
Ana Dias-Curado 1, Carolina Amaro-Gonçalves 2, Maria João-Palaré 3, Filipa Oliveira-Ramos 4, Anabela Ferrão 3
1 Department of Pediatrics, Hospital de Santa Maria Centro Hospitalar Universitário Lisboa Norte, Lisboa, Portugal; 2 Department of Pediatrics, Unidade Local de Saúde de Santa Maria. Lisbon, Portugal; 3 Pediatric Hematology Unit, Department of Pediatrics, Hospital de Santa Maria; Clínica Universitária de Pediatria, Faculdade de Medicina, Universidade de Lisboa; Lisboa, Portugal; 4 Pediatric Rheumatology Unit, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte; Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa. Lisboa, Portugal
Ana Dias-Curado, Carolina Amaro-Gonçalves, Maria João-Palaré, Filipa Oliveira-Ramos, Anabela Ferrão
La información completa de afiliaciones y autor de correspondencia está disponible en la versión original en PDF.
*Correspondence: Ana Dias-Curado, Email not available
Abstract
Antiphospholipid syndrome (APS) is an autoimmune systemic disorder characterized by an increased risk of thrombosis and the presence of circulating antiphospholipid antibodies (aPL). A previously healthy 7-year-old girl was admitted complaining of right leg and foot pain with claudication. She had a malar rash, pallor and cooling of the limb, cyanosis of the toes and non-palpable popliteal and distal pulses. The vascular doppler showed a right popliteal artery occlusion and she started enoxaparin. Laboratory studies showed positive Coombs test, triple positive aPL, positive antinuclear and anti-double stranded DNA antibodies, C4 mild consumption. She started hydroxychloroquine, acetylsalicylic acid and warfarin, with posterior suspension of enoxaparin. Diagnosis of APS was confirmed 12 weeks later as aPL remained positive. There was a good clinical evolution with signs of re-vascularization. APS is rare in the pediatric population and has a wide clinical spectrum, becoming a diagnostic and therapeutic challenge due to the small number of pediatric studies.
