Theron Alexandre, Biron-Andreani Christine, Jeziorski Eric
Department of Pediatric Oncology and Hematology, Univ Montpellier, CHU Montpellier, Montpellier, France.
Resources and Competence Center for Constitutional Bleeding Disorders, Univ Montpellier, CHU Montpellier, Montpellier, France.
Acta Paediatr. 2025 Oct;114(10):2511-2517. doi: 10.1111/apa.70193. Epub 2025 Jun 23.
Acquired Idiopathic purpura fulminans is a rare pathology caused by anti-protein S antibodies. We have conducted a literature review focusing on the most recent developments in diagnosis and management. Diagnosis is based on the demonstration of typical necrotic purpura lesions on the legs. Treatment must be initiated without delay by infusion of fresh frozen plasma, plasmapheresis, or infusion of immunoglobulins, and curative anticoagulation. The challenge of limiting the incidence of serious complications can be met by disseminating simple diagnostic and treatment keys to paediatricians, with particular emphasis on recognising typical skin lesions.
获得性特发性暴发性紫癜是一种由抗蛋白S抗体引起的罕见病症。我们进行了一项文献综述,重点关注诊断和治疗方面的最新进展。诊断基于腿部典型坏死性紫癜病变的表现。必须立即通过输注新鲜冷冻血浆、血浆置换或输注免疫球蛋白以及进行根治性抗凝来开始治疗。通过向儿科医生传播简单的诊断和治疗要点,特别是强调识别典型皮肤病变,可以应对限制严重并发症发生率的挑战。