PDF(Pubmed)
Abstract：
A diagnosis of dermatomyositis requires recognition of distinct patterns of skin disease in combination with, and sometimes without, muscle weakness. Often, a striking contrast between involved and uninvolved areas is observed. Familiar patterns include eyelid and midfacial eruptions, Gottron papules/sign, and upper back (shawl sign), central chest (V/open collar sign), and lateral thigh (holster sign) involvement. More recently, new specific antibody/phenotype-associated patterns have been reported. We describe a case series of two distinct patterns of skin involvement in six adult patients with both classical and amyopathic dermatomyositis. Three had paraneoplastic disease. All had intermediate to richly pigmented skin; five were of Afro-Caribbean and one was of Asian-Caribbean descent. Four were men, and two were women. Ages ranged from 41 to 89 years. All patients had concomitant hallmark signs (facial, hand, and/or trunk signs). Three were amyopathic. The first pattern involved a sharply demarcated, horizontally oriented hyperpigmented patch/thin plaque across the shoulders and upper chest, extending up the anterior neck. The second was the combination of the classical upper back shawl distribution with distinct mid-back sparing and diffuse involvement of the lower back. Named patterns help with the recognition of skin rashes in dermatomyositis. Based on the current lexicon describing items of apparel, we liken the first pattern to a \"fur stole and turtleneck\" sign and the latter to a \"halter-back\" or \"reflected-shawl\" sign. Biopsies revealed hyperkeratosis and interface dermatitis, often with epidermal atrophy, compatible with dermatomyositis. These patterns perhaps represent the coalescence of already well-described signs, photo-exacerbation, koebnerization, mechanical stretch, and other currently unclear factors contributing to patterning in dermatomyositis. Pattern distribution recognition is particularly valuable in individuals with richly pigmented skin who may lack typical violaceous erythema. The distinct demarcation led to the initial misdiagnosis of allergic contact dermatitis or other exogenous dermatitis in most of our patients. Further work involves evaluation of antibody phenotype and internal involvement associations. Limitations include lack of specific antibody panels and longitudinal follow-up data.
摘要：
皮肌炎的诊断需要识别皮肤疾病的不同模式，有时没有，肌肉无力。通常,在涉及的和未涉及的区域之间观察到明显的对比。熟悉的模式包括眼睑和面部中部出疹，Gottron丘疹/体征,和上背部（披肩标志），中央胸部（V/开领标志），大腿外侧(皮套征)受累。最近,已经报道了新的特异性抗体/表型相关模式。我们描述了六名患有经典和肌病性皮肌炎的成年患者的两种不同皮肤受累方式的病例系列。三人患有副肿瘤疾病。所有人都有中等至丰富的色素皮肤；五个是非洲裔加勒比人，一个是亚洲裔加勒比人。四个是男人，还有两个是女人.年龄从41岁到89岁不等。所有患者都有伴随的标志体征（面部，手,和/或树干标志)。三个是肌病。第一个模式涉及一个清晰的划界，水平取向的色素沉着过度斑块/薄斑块横跨肩部和上胸部，延伸到前颈.第二个是经典的上背部披肩分布与明显的中背部保留和下背部的弥散参与相结合。命名模式有助于识别皮肌炎中的皮疹。根据当前描述服装物品的词典，我们将第一个图案比作“皮草和高领毛衣”标志，后者比作“背背”或“反射披肩”标志。活检显示角化过度和界面皮炎，通常有表皮萎缩，与皮肌炎相容。这些模式也许代表了已经很好描述的迹象的结合，照片恶化，koebnerization，机械拉伸,以及其他目前尚不清楚的导致皮肌炎模式的因素。在可能缺乏典型的紫色性红斑的皮肤色素丰富的个体中，模式识别特别有价值。明显的分界导致我们大多数患者最初误诊为过敏性接触性皮炎或其他外源性皮炎。进一步的工作涉及抗体表型和内部参与关联的评估。局限性包括缺乏特异性抗体组和纵向随访数据。
