Bartl Mery, Gomez Casanovas Jose G, Loftis Christine E, Rincon-Rueda Laura, Suarez Parraga Andres R
Internal Medicine, University of Texas Rio Grande Valley School of Medicine, Edinburg, USA.
Internal Medicine, University of Texas Rio Grande Valley School of Medicine, McAllen, USA.
Cureus. 2023 May 26;15(5):e39540. doi: 10.7759/cureus.39540. eCollection 2023 May.
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.
皮肌炎（DM）是一种罕见的全身性自身免疫性疾病，与皮肤和肌肉的炎症相关。其典型表现为近端肌肉无力，伴有如Gottron丘疹和向阳疹等特征性皮肤病变。该疾病最可怕的并发症之一是自发性出血性肌炎的出现，因为大多数报告病例是致命的。这种情况的机制或危险因素尚未阐明；然而，在先前的病例报告中，预防性抗凝与之相关，尽管也可能存在特发性出血性肌炎。我们报告一例近期诊断为皮肌炎患者的自发性肌肉内出血（SIH）病例。一名59岁的西班牙裔男性患者，有近期诊断的前列腺癌和皮肌炎病史，因贫血加重就诊于急诊科（ED）。他之前的血红蛋白（Hgb）为9 g/dl，但重复实验室检查显示在急诊科时降至6.5 g/dl，随后又降至5.5 g/dl。入院时，患者无发热，心动过速，血压正常，无明显胃肠道出血迹象。体格检查发现右侧大腿内侧有瘀斑，直肠指检阴性。由于怀疑腹膜后血肿，遂进行了腹部和盆腔的非增强计算机断层扫描（CT），结果显示右侧腹股沟出现了一个最大直径达6 cm的复杂液性聚集区，考虑可能为血肿。该患者之前在该区域未进行过任何血管手术，但在上次住院期间接受了深静脉血栓形成（DVT）预防措施。咨询血管外科后，建议进行保守治疗。第三天，患者出现新发的左侧胸膜炎性胸痛。检查时，发现其左侧胸肌有明显肿胀和压痛，入院时并无此症状。由于担心潜在血肿，遂进行了胸部非增强CT检查，结果显示双侧胸肌增厚，右侧更明显，有一个2.5 cm×1.3 cm的液性聚集区。此外，右侧胸壁外侧肌肉、右后侧斜方肌或冈上肌增厚，很可能是由于肌肉内出血所致。患者被转至二级护理病房进行密切监测。继续进行保守治疗，根据需要进行输血，持续三天，直到血红蛋白稳定在9.8 mg/dL。病情稳定后，患者重新开始使用类固醇和免疫抑制治疗，随后SIH症状消退。SIH在皮肌炎中已有报道，特别是在那些抗MDA - 5抗体阳性的患者中更为突出。一项病例系列研究和文献综述显示，出现SIH的患者在六个月内死亡率为60.9%，深部肌肉出血患者的预后较差（死亡率80%），而浅表出血患者的死亡率为25%。目前对于治疗方法尚无共识，且动脉栓塞尚未被证明有效。在我们的患者中，通过密切监测和频繁输血的保守治疗有助于实现血流动力学稳定。临床医生应更加关注皮肌炎患者中这些罕见但危及生命的并发症。
