A Case of Hemorrhagic Myositis Associated With Prophylactic Heparin Use in Dermatomyositis

Abstract

Dermatomyositis (DM) is a rare systemic autoimmune disease that is associated with inflammation of the skin and muscles. It typically presents with weakness of the proximal muscles along with characteristic skin lesions such as Gottron's papules and heliotrope rash. One of the most feared complications of this disease is the appearance of spontaneous hemorrhagic myositis, as most reported cases are fatal. The mechanism or risk factors of this condition have not been elucidated; however, prophylactic anticoagulation has been correlated with it in previous case reports, although idiopathic hemorrhagic myositis may also be present. We present a case of spontaneous intramuscular hemorrhage (SIH) in a recently diagnosed DM patient. A 59-year-old Hispanic male with a medical history of recently diagnosed prostate cancer and DM presented to the emergency department (ED) due to worsening anemia. His previous hemoglobin (Hgb) was 9 g/dl, but repeated laboratory tests revealed a level of 6.5 g/dl and later 5.5 g/dl at the ED. On admission, the patient was afebrile, tachycardic, and normotensive, with no overt sign of gastrointestinal bleeding. The physical exam revealed an ecchymosis on the right medial aspect of the thigh, and a digital rectal exam was negative. Computer tomography (CT) of the abdomen and pelvis without contrast was ordered due to suspicion of a retroperitoneal hematoma, revealing an interval development of a right groin complex fluid collection of up to 6 cm, concerning a possible hematoma. The patient did not have any previous vascular procedures in the area but was exposed to deep vein thrombosis (DVT) prophylaxis during the previous admission. Vascular surgery was consulted, and the recommendation was made to proceed with conservative management. On the third day, the patient developed new-onset, left-sided pleuritic chest pain. Upon examination, significant swelling and tenderness were noted in his left pectoral region, which was not present on admission. A CT chest without contrast was ordered due to concerns of underlying hematomas, revealing bilateral thickening of the pectoralis muscles, more on the right side, with a fluid collection of 2.5 cm × 1.3 cm. In addition, there was thickening of the right lateral chest wall muscles in the posterior right trapezius or supraspinatus muscles, most likely from intramuscular hemorrhage. The patient was transferred to the step-down unit for close monitoring. Conservative management was continued with as-needed transfusions for three days until hemoglobin stabilized at 9.8 mg/dL. Once stable, the patient was resumed on steroids and immunosuppressive therapy with posterior resolution of the SIH. SIH has been reported in DM, particularly more prominent in those with anti-MDA-5 antibodies. A case series and literature review showed 60.9% mortality within six months in those presenting with SIH, with a poorer prognosis (80% mortality) in those with deep muscle bleeding versus superficial (25%). There is currently no consensus on the treatment approach, and arterial embolization has not been proven effective. In our patient, conservative management with close surveillance and frequent transfusions helped achieve hemodynamic stability. Clinicians should be more aware of these rare but life-threatening complications in patients presenting with DM.