[The 521st case: fever, livedo reticularis, cerebral venous and sinus thrombosis, intracerebral hemorrhage].

Authors

C S Chen, J Y Dai, M Shen

Published in

Zhonghua nei ke za zhi. Volume 65. Issue 10. Pages 1134-1138. Oct 01, 2026.

Abstract

A 14-year-old male was admitted with a three-month history of fever and eyelid erythema and edema. The clinical course was notable for progressive weight loss, livedo reticularis, and multiple lymph node enlargements. Laboratory investigations revealed negative antinuclear antibodies,significantly reduced complement levels, positive anticardiolipin antibodies, hemolytic anemia, and proteinuria. Imaging studies demonstrated pulmonary inflammation, intracranial venous sinus thrombosis, and pericardial effusion. Based on a comprehensive assessment, the patient was diagnosed with systemic lupus erythematosus (antinuclear antibodies-negative), complicated by antiphospholipid syndrome and cerebral venous sinus thrombosis. Initial management included pulse steroid therapy, intravenous immunoglobulin, immunosuppressive agents, and heparin anticoagulation, which resulted in transient clinical improvement. During hospitalization, the patient developed acute intracranial hemorrhage, necessitating emergent surgical decompression and therapeutic adjustment. Subsequently, an active pulmonary infection emerged and was treated with antimicrobial therapy alongside Pneumocystis jirovecii pneumonia prophylaxis, leading to stabilization of his condition.This case underscores several key considerations: (1) the diagnostic complexity of childhood SLE, particularly in ANA-negative cases, which requires integration of clinical and extensive immunologic profiling; (2) the importance of testing for non-criteria antiphospholipid antibodies in suspected antiphospholipid syndrome; (3) the fact that cerebral venous sinus thrombosis is a rare but severe complication in which anticoagulation must be carefully balanced against the risk of hemorrhage; and (4) the critical need for vigilant infection prevention, proactive management, and close monitoring of drug-related toxicities. A multidisciplinary approach is essential to tailor individualized treatment strategies and optimize patient outcomes.

PMID:
42855764
Bibliographic data and abstract were imported from PubMed on 10 Oct 2026.

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