关键词: anti-golgi apparatus ab anti-pm/scl ab hypomyopathic dermatomyositis low-dose methotrexate normal cpk overlap syndromes palmoplantar keratoderma topical tacrolimus 0.1%

来  源:   DOI:10.7759/cureus.56531   PDF(Pubmed)

Abstract:
Overlap syndrome is a clinical challenge and brings together a wide range of treatment options for the treating physician. Addressing each and every complaint of the patient is crucial. A 50-year-old female patient presented with skin thickening, blackening, and hyperkeratosis; dysphagia; joint pain; features of myopathy; Raynaud\'s phenomenon; and dry mouth. Inflammatory markers were raised along with a positive antinuclear antibody (ANA) with Golgi apparatus pattern, anti-Sjögren\'s-syndrome-related antigen A (anti-SSA)/Ro60 3+, anti-SSA/Ro52 3+, and anti-PM/Scl 2+ antibodies that suggested overlap syndrome. Although the patient had no respiratory complaints, a unique interstitial lung disease (ILD) pattern was noted during the evaluation. Skin manifestations were puzzling, but the histopathology analyses of skin biopsies taken from two different sites revealed distinguishing features of cutaneous lupus and dermatomyositis. Treatment with hydroxychloroquine, pilocarpine, nifedipine, methotrexate, and topical tacrolimus produced a dramatic improvement in the clinical features. This case highlights subtle and florid features of different autoimmune diseases. The hyperkeratotic skin changes were the most striking feature, but the whole evaluation process unveiled many rare presentations of known autoimmune conditions that can open doors to new areas of our understanding toward connective tissue diseases (CTDs). Our case report demonstrates significant heterogeneity in the ANA patterns, ILD patterns, clinical manifestations, and treatment approaches.
摘要:
重叠综合征是一项临床挑战,为治疗医师带来了广泛的治疗选择。解决患者的每一个投诉至关重要。一名50岁的女性患者出现皮肤增厚,变黑,角化过度;吞咽困难;关节痛;肌病的特征;雷诺现象;和口干。炎症标志物随着高尔基体模式的抗核抗体(ANA)阳性而升高,抗干燥综合征相关抗原A(抗SSA)/Ro603+,抗SSA/Ro523+,和提示重叠综合征的抗PM/Scl2+抗体。尽管患者没有呼吸道疾病,在评估过程中发现了一种独特的间质性肺病(ILD)模式.皮肤表现令人费解,但对来自两个不同部位的皮肤活检的组织病理学分析显示了皮肤狼疮和皮肌炎的显著特征。用羟氯喹治疗,毛果芸香碱,硝苯地平,甲氨蝶呤,和外用他克莫司在临床特征上产生了显著的改善。此病例突出了不同自身免疫性疾病的微妙和花哨的特征。角化过度的皮肤变化是最显著的特征,但整个评估过程揭示了已知自身免疫性疾病的许多罕见表现,这些表现可以为我们对结缔组织疾病(CTDs)的新认识领域打开大门.我们的病例报告显示了ANA模式的显著异质性,ILD模式,临床表现,和治疗方法。
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