Saoud Bouchra, Allali Fadoua, Hassouni Najia Hajjaj
Rheumatology B Department, El Ayachi Hospital, Rabat-Salé Teaching Hospitals, Morocco.
Joint Bone Spine. 2006 May;73(3):318-20. doi: 10.1016/j.jbspin.2005.05.004. Epub 2005 Sep 16.
Amyopathic dermatomyositis (ADM) is a rare condition characterized by skin lesions suggestive of dermatomyositis (DM) without detectable muscle abnormalities after at least 2 years of follow-up. Pulmonary fibrosis is uncommon in patients with ADM.
A 64-year-old woman presented with a 2 years and 6 months history of nondestructive polyarthritis. She had skin changes suggestive of DM, including a pink rash over the face, neck, and forearms; Gottron's papules over the metacarpophalangeal joints; and heliotrope edema of the eyelids. She reported no muscle symptoms. Findings were normal from muscle enzyme assays, electromyography, and muscle biopsies. A diagnosis of ADM was given. Early lung fibrosis was found. Investigations for a tumor were negative.
ADM is a rare condition that may be an abortive form of DM with a favorable outcome and a lower risk of malignancy compared to classic DM. However, the development of pulmonary fibrosis may cloud the prognosis.
无肌病性皮肌炎(ADM)是一种罕见疾病,其特征为出现提示皮肌炎(DM)的皮肤病变,且至少经过2年随访未发现可检测到的肌肉异常。肺纤维化在ADM患者中并不常见。
一名64岁女性,有2年6个月的非破坏性多关节炎病史。她有提示DM的皮肤改变,包括面部、颈部和前臂的粉红色皮疹;掌指关节处的Gottron丘疹;以及眼睑的向阳疹水肿。她未报告肌肉症状。肌肉酶检测、肌电图和肌肉活检结果均正常。诊断为ADM。发现早期肺纤维化。肿瘤检查结果为阴性。
ADM是一种罕见疾病,可能是DM的一种顿挫型,与经典DM相比,预后良好且恶性风险较低。然而,肺纤维化的发展可能会影响预后。