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Background: Spontaneous hemorrhage is a rare but serious complication of dermatomyositis (DM).
Methods: We report a 61-year-old man with anti-NXP2-positive DM who developed a retroperitoneal hematoma without anticoagulation, shortly after corticosteroid initiation.
Results: Laboratory investigations demonstrated rhabdomyolysis, hepatic injury and coagulopathy. The patient was managed with red blood cell transfusion, factor VIII, high-dose corticosteroids and intravenous immunoglobulin, resulting in complete recovery.
Conclusions: Although hemorrhagic events in DM are most often linked to anti-MDA5 or anti-Ro52 antibodies, their association with anti-NXP2 antibodies remains uncertain. This case highlights the importance of early recognition and prompt intervention in managing spontaneous hemorrhage in DM.
DOI: 10.7754/Clin.Lab.2025.250919
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