Auriemma M, Capo A, Meogrossi G, Amerio P
Department of Medicine and Aging Science Dermatologic Clinic G. d'Annunzio University of Chieti‑Pescara, Italy -
G Ital Dermatol Venereol. 2014 Oct;149(5):505-17. Epub 2014 Jul 11.
Idiopathic immune myopathies (IIM) are an heterogeneous group of autoimmune muscle disorders characterized by progressive muscle involvement. Dermatomyositis (DM) is the most common form of IIM. It is a multisystem disorder characterized by symmetric proximal, extensor, inflammatory myopathy, vascular involvement and a characteristic cutaneous eruption. Six types of DM have been identified: idiopathic, juvenile (JDM), cancer-related other autoimmune diseases-related, iatrogenic DM and amyopathic DM. Cutaneous manifestations of DM are the most important aspect of this disease and can precede from several months to years muscle or systemic involvement. Three groups of signs have been described: pathognomonic, highly characteristic and compatible. Although differences exist among the different clinical presentation of skin lesions, they share common histological findings including the presence of interface dermatitis with epidermal atrophy, basement membrane degeneration, vacuolar alteration of basal keratinocytes, and dermal changes consisting of interstitial mucin deposition and a sparse lymphocytic infiltrate. DM is a serious disease; the correct evaluation of any skin lesion suggesting an early diagnosis is of utmost importance. Skin signs may, also, represent a marker of treatment efficacy even though systemic symptoms worsening may not always be followed by more severe skin lesions.
特发性免疫性肌病（IIM）是一组异质性自身免疫性肌肉疾病，其特征为进行性肌肉受累。皮肌炎（DM）是IIM最常见的形式。它是一种多系统疾病，其特征为对称性近端、伸肌、炎性肌病、血管受累以及特征性皮疹。已确定六种类型的DM：特发性、青少年型（JDM）、癌症相关型、其他自身免疫性疾病相关型、医源性DM和无肌病性DM。DM的皮肤表现是该疾病最重要的方面，可在肌肉或全身受累前数月至数年出现。已描述了三组体征：特异性体征、高度特征性体征和符合性体征。尽管皮肤病变的不同临床表现存在差异，但它们具有共同的组织学表现，包括界面性皮炎伴表皮萎缩、基底膜变性、基底角质形成细胞空泡改变，以及由间质粘蛋白沉积和稀疏淋巴细胞浸润组成的真皮改变。DM是一种严重疾病；正确评估任何提示早期诊断的皮肤病变至关重要。皮肤体征也可能代表治疗效果的指标，即使全身症状恶化并不总是伴随着更严重的皮肤病变。
