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Seven-year itch: a perplexing case of lichen planus-lupus erythematosus overlap syndrome

机译:七年之痒:扁平苔藓-红斑狼疮重叠综合征的一例令人困惑的病例

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Lichen planus-lupus erythematosus overlap syndrome is a rare disorder characterized by clinical and histopathological features of both lichen planus (LP) and lupus erythematosus (LE). Cutaneous lesions commonly affect the distal arms, legs, face, and trunk and these plaques are often large, scaly, painful, and atrophic, often exhibiting hypopigmentation or a red to blue-violet color. We report a case of LP-LE overlap syndrome diagnosed in a man previously believed to have atypical lichen planus who presented with an exacerbation of exuberant pruritic erythematous scaly plaques. The patient had six separate skin biopsies all of which displayed features of LP. Because the clinical symptoms did not correlate to the histopathological picture, a seventh skin biopsy with direct immunofluorescence (DIF) was performed and immunologic markers measured. The DIF demonstrated early lupus bands; serologic testing exhibited elevated ANA and anti-SSA. These findings established the diagnosis of LP-LE overlap syndrome. The patient was started on hydroxychloroquine with short-term trials of oral prednisone during disease flares, which took place in the first three months of treatment.
机译:扁平苔藓-红斑狼疮重叠综合征是一种罕见疾病,其特征在于扁平苔藓(LP)和红斑狼疮(LE)的临床和组织病理学特征。皮肤病变通常会影响远端的手臂,腿,面部和躯干,这些斑块通常较大,鳞片状,疼痛和萎缩,通常表现为色素沉着不足或呈红色至蓝紫色。我们报告一例LP-LE重叠综合征的诊断,该患者先前被认为患有非典型的扁平苔藓,并伴有旺盛的瘙痒性红斑鳞片斑块。该患者进行了六次单独的皮肤活检,均显示了LP的特征。由于临床症状与组织病理学图像不相关,因此进行了第七次直接免疫荧光(DIF)皮肤活检并测量了免疫标记。 DIF证实了早期的狼疮带。血清学检查显示ANA和抗SSA升高。这些发现确定了LP-LE重叠综合征的诊断。在疾病发作期间对患者进行口服泼尼松的短期试验,开始使用羟氯喹治疗,该试验在治疗的前三个月进行。

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