Spontaneous Retroperitoneal Hemorrhage in Anti-NXP2-Positive Dermatomyositis: a Rare but Severe Complication.

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.