Mast cells and type I interferon responses in the skin of patients with juvenile dermatomyositis: are current therapies just scratching the surface?
Mast cells and type I interferon responses in the skin of patients with juvenile dermatomyositis: are current therapies just scratching the surface?
复制标题
幼年皮肌炎患者皮肤中的肥大细胞和 I 型干扰素反应：当前的治疗方法只是触及表面吗？
DOI：
10.1002/art.27525
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发表时间：
2010
影响因子：
--
通讯作者：
Miller,FrederickW
中科院分区：
文献类型：
--
作者：
Rider,LisaG;Miller,FrederickW
皮肌炎（DM）是一种全身性自身免疫性疾病，其特征是近端骨骼肌、皮肤和其他靶器官的慢性炎症。许多医生关注的弱点和由此产生的身体功能障碍的最禁用功能的糖尿病和治疗试验往往是针对改善这些表现。随后，皮肤病往往被治疗医生忽视，作为一种不太严重的疾病表现，往往不是治疗的重点。然而，尽管二手状态往往提供皮肤病的DM，它可以作为衰弱的肌肉疾病。成人和青少年糖尿病患者经常出现各种日光暴露和非日光暴露皮疹，包括皮疹和血管病变（1; 2）。瘙痒是活动性DM皮肤病的常见表现，对生活质量产生不利影响（3）。治疗不彻底的皮肤表现可导致多达25%的青少年发病的DM患者和15%的成年DM患者的皮肤和皮下组织中的营养不良性钙化（4）。皮肤萎缩、色素沉着和脂肪营养不良、皮下组织缺失导致胰岛素抵抗和高脂血症的代谢后遗症，是皮肤和皮下组织既往炎症的常见结局（4; 5）。
Dermatomyositis (DM) is a systemic autoimmune disease characterized by chronic inflammation in proximal skeletal muscles, skin and other target organs. Many physicians focus on the weakness and the resulting physical dysfunction as the most disabling features of DM and therapeutic trials often have been directed at improving these manifestations. Subsequently, the skin disease is often neglected by treating physicians as a less severe manifestation of illness and is often not the focus of therapy.Nonetheless, despite the second-hand status often afforded the skin disease of DM, it can be as debilitating as the muscle disease. Patients with adult and juvenile DM often experience a variety of both sun-exposed and non-sun-exposed skin rashes, including erythematous and vasculopathic lesions (1; 2). Pruritus is a frequent manifestation of active DM skin disease that adversely impacts quality of life (3). Incompletely treated cutaneous manifestations can lead to dystrophic calcification in the skin and subcutaneous tissues in up to 25% of juvenile-onset DM patients and 15% of adults with DM (4). Skin atrophy, dyspigmentation and lipodystrophy, the loss of subcutaneous tissue resulting in metabolic sequelae of insulin resistance and hyperlipidemia, are common outcomes of prior inflammation in the skin and subcutaneous tissue (4; 5).