Ismail Awab, Moiddin Nekhal M, Mahmood Mohammed A, Khan Asad Ullah, Nagaraj Neha
Internal Medicine, Queen Elizabeth Hospital Birmingham, Birmingham, GBR.
Acute Internal Medicine, Good Hope Hospital, Birmingham, GBR.
Cureus. 2024 Nov 6;16(11):e73154. doi: 10.7759/cureus.73154. eCollection 2024 Nov.
Dermatomyositis is a multi-system, connective tissue immune-mediated inflammatory condition characterised by myositis and distinct skin manifestations, with a higher prevalence in women. Symptoms typically appear in adulthood, though a juvenile form exists. Early signs may include Gottron's papules and heliotrope rash, with proximal muscle weakness as the second most common initial symptom. This report discusses a case of a 55-year-old woman whose presentation initially resembled acute demyelinating polyneuropathy (ADP), beginning with an eight-week history of dry cough and a five-day history of diarrhoea for four weeks followed by muscle weakness and no skin signs. The patient, who had delayed medical attention, presented with significant proximal muscle weakness and absent reflexes, resulting in a debilitating reduction in her baseline mobility. Initial investigations that were conducted included an elevated creatinine kinase (CK) level and reduced forced vital capacity (FVC). Her cerebrospinal fluid (CSF) analysis showed elevated proteins. She was admitted to the intensive care unit due to a declining FVC, and receiving intravenous immunoglobulin (IVIG), which resulted in some improvement. Over time, she developed skin manifestations and responded to treatment with mycophenolate mofetil (MMF) and corticosteroids.
皮肌炎是一种多系统、结缔组织免疫介导的炎症性疾病,其特征为肌炎和独特的皮肤表现,女性患病率较高。症状通常在成年期出现,不过也有青少年型。早期体征可能包括Gottron丘疹和向阳疹,近端肌无力是第二常见的初始症状。本报告讨论了一例55岁女性病例,其最初表现类似急性脱髓鞘性多发性神经病(ADP),起初有8周干咳病史、4周腹泻5天病史,随后出现肌无力且无皮肤体征。该患者延误了就医,出现明显的近端肌无力且反射消失,导致其基线活动能力严重下降。进行的初始检查包括肌酸激酶(CK)水平升高和用力肺活量(FVC)降低。她的脑脊液(CSF)分析显示蛋白质升高。由于FVC下降,她被收入重症监护病房,并接受静脉注射免疫球蛋白(IVIG)治疗,病情有所改善。随着时间推移,她出现了皮肤表现,并对霉酚酸酯(MMF)和皮质类固醇治疗有反应。