| 제목 |
A Rare Presentation of Inflammatory Myositis as a Menifestation of Morphea: A Case Report |
| 소속 |
Samsung Medical Center, Department of Rehabilitation Medicine1, Samsung Medical Center, Department of Dermatology2 |
| 저자 |
Kyung Ah Kim1*, Jae Lim Kim1, Dong-Youn Lee2, Duk Hyun Sung1† |
Decription
A 50-year-old Korean woman presented to the locomotor clinic of the department of Physical Medicine and Rehabilitation with a six-year history of progressive weakness and sclerotic skin changes. She couldn’t climb stairs without holding handrails, had difficulty with raising hands above her head since 2012. On examination, both proximal upper and lower extremity weakness, decreased range of motion of both shoulders, both hip contractures were noticed. When palpated, both thigh muscle hardening was present. Discolored sclerotic skin lesions were found on her anterior neck, chest, abdomen and low back. There were no evidences of sclerodactyly, capillary change of nailbed, and Raynaud phenomenon. In laboratory tests, Anti-SSA was the only positive auto antibody. Nerve conduction study was normal but EMG showed denervation potentials and early MUAP recruitment in both upper and lower extremity proximal muscles. The skin biopsy did not have morphea like features. Lower extremity MRI showed diffuse enhancement, muscle edema, fascia thickening of both hip and thigh muscles. Muscle biopsy from vastus lateralis resembled inflammatory myopathy. Her symptoms and clinical evaluations gave the impression of generalized morphea with muscle involvement rather than systemic sclerosis or polymyositis. The patient was managed with oral prednisolone. After 40days of oral prednisolone treatment, the patient showed improvement in anterior neck flexor muscle strength, sclerotic skin lesions and serum CK level. However, weakness of both proximal upper and lower extremity persisted.
Conclusion
Morphea is an autoimmune skin disorder characterized by inflammation and sclerosis of the skin and soft tissue. It is often misdiagnosed or not timely treated since its rare incidence rate, feature shared with systemic sclerosis and possibility of overlapping with other autoimmune disease. A clinician should keep in mind that myositis can be one of morphea’s systemic presentation and should be able to confirm the diagnosis and initiate appropriate evaluation and treatment.
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Both lower extremity weakness, muscle atrophy, both hip contracture, hand on thigh posture while standing were observed.
Discolored sclerotic skin lesions were found on her anterior neck, chest, abdomen and lower back.
Abnormal high signals in muscles and fasciae of pelvic girdle and proximal thigh representing acute stage inflammatory myofascitis.
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