국소성 경피증 및 전신성 경피증 양 질환에 관한 고찰

Abstract

Objective: Systemic sclerosis is a rare connective tissue disorder characterized by skin fibrosis, obliterative vasculopathy and distinct autoimmune abnormalities. However, many other clinical conditions present with substantial skin fibrosis and may be confused with systemic sclerosis, leading to a misdiagnosis. Localized scleroderma (morphea) is a rare skin-limited autoimmune disease characterized by cutaneous fibrosing and obstructive vasculopathy. This disease may invade into subcutaneous fat layer and provoke permanent functional disability. Scleroderma-like disorder presents with substantial skin fibrosis and may be confused with systemic sclerosis, sometimes leading to an incorrect diagnosis. Because of its rarity, there have been few clinical surveys of patients with localized scleroderma and scleroderma-like disorders in Korea. The aim of this study was to elucidate the clinical presentation, serological data, and clinical outcomes of localized scleroderma and scleroderma-like disorder. Methods: The clinical survey was retrospectively performed through the available medical records during a 7-year-period from 2004 to 2010, in 43 cases with localized scleroderma and 4 cases with scleroderma-like disorder. Results: Localized scleroderma occurred primarily in females (female to male ratio being 2.6:1). Most patients were between 10 to 29 years of age. The mean age at diagnosis was 26.2 years. Plaque (51.2%) and linear morphea (37.2%) were most common. No case was associated with systemic sclerosis. The most common site of plaque morphea was trunk (47.8%). In linear type, the most commonly occurring lesions were head-neck (52.9%). The FANA was positive in 23.3% of all cases. Treatment included systemic corticosteroid, colchicine, anti-malarial agents, D-penicillamine or intralesional triamcinolone injection. Clinical improvement, including both significant and partial remission, was demonstrated in only 62.8% of the treated cases. Additionally, we experienced 4 cases of scleroderma-like disorder which did not correspond to criteria of other scleroderma. Conclusion: Localized scleroderma is a chronic inflammatory skin-limited disease without potential risk of evolving into systemic sclerosis. In order to exclude other diseases, thorough investigation including a history-taking, physical examination, serologic studies, and histopathologic examinations should be conducted.