Sunny Neethu, Kashyap Kritartha, Kumar Arjun, Parchani Ashwin, Dhar Minakshi
Geriatrics, All India Institute of Medical Sciences, Rishikesh, IND.
Internal Medicine, All India Institute of Medical Sciences, Rishikesh, IND.
Cureus. 2023 May 11;15(5):e38895. doi: 10.7759/cureus.38895. eCollection 2023 May.
Dermatomyositis (DM) is a systemic autoimmune disease that primarily affects the skin and muscles. The hallmark skin manifestation is a violaceous rash on the face, neck, shoulders, upper chest, and extensor surfaces of the arms and legs, which is often accompanied by edema and can be exacerbated by exposure to sunlight. Generalized limb edema and dysphagia are rare presentations of dermatomyositis. Here we present a case of a 69-year-old woman presenting with generalized limb swelling, periorbital swelling, and dysphagia which was diagnosed as dermatomyositis based on a combination of clinical, laboratory, and imaging findings. The patient had an absence of complaints of limb weakness and a predominance of complaints of edema and dysphagia which posed a diagnostic challenge. The patient was treated with high-dose steroids and immunosuppressive therapy, leading to a significant improvement in her symptoms. Edematous dermatomyositis has been associated with underlying malignancy in 25% of the cases and this warrants close follow-up and malignancy screening for such patients. In some cases, subcutaneous edema might be the only manifestation of the disease. This case underscores the importance of recognizing DM as a potential differential diagnosis in patients presenting with generalized edema and dysphagia, particularly in the initial absence of classic skin findings. This rare presentation of dermatomyositis may be a hallmark of a severe form of the disease and requires prompt recognition and aggressive treatment.
皮肌炎(DM)是一种主要累及皮肤和肌肉的系统性自身免疫性疾病。其标志性的皮肤表现为面部、颈部、肩部、上胸部以及手臂和腿部伸侧出现紫红色皮疹,常伴有水肿,且暴露于阳光下会加重。全身性肢体水肿和吞咽困难是皮肌炎罕见的表现形式。在此,我们报告一例69岁女性患者,其表现为全身性肢体肿胀、眶周肿胀和吞咽困难,根据临床、实验室及影像学检查结果综合诊断为皮肌炎。该患者无肢体无力主诉,主要为水肿和吞咽困难主诉,这给诊断带来了挑战。患者接受了大剂量类固醇和免疫抑制治疗,症状有显著改善。水肿性皮肌炎在25%的病例中与潜在恶性肿瘤相关,因此对此类患者需要密切随访和进行恶性肿瘤筛查。在某些情况下,皮下水肿可能是该病的唯一表现。该病例强调了在出现全身性水肿和吞咽困难的患者中,尤其是最初无典型皮肤表现时,将皮肌炎作为潜在鉴别诊断的重要性。这种皮肌炎的罕见表现可能是该病严重形式的一个标志,需要及时识别并积极治疗。