AMYOPATHIC DERMATOMYOSITIS (DERMATOMYOSITIS SINE MYOSITIS) - PRESENTATION OF 6 NEW CASES AND REVIEW OF THE LITERATURE
AMYOPATHIC DERMATOMYOSITIS (DERMATOMYOSITIS SINE MYOSITIS) - PRESENTATION OF 6 NEW CASES AND REVIEW OF THE LITERATURE
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DOI：
10.1016/0190-9622(91)70153-s
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发表时间：
1991-06-01
影响因子：
13.8
通讯作者：
SONTHEIMER, RD
中科院分区：
文献类型：
--
作者：
EUWER, RL;SONTHEIMER, RD
我们报告了6例皮肌炎典型皮肤表现的患者，他们在出现皮肤表现至少2年后没有出现肌肉疾病的临床或实验室证据。这类患者占我们15年期间皮肌炎患者总经验的11%。所有六个病人Gottron鲍里斯,periungual红斑/毛细管扩张,和紫罗兰色的变色的脸,脖子,胸部,在一些时间在他们的疾病。此外，所有患者均有瘙痒和光敏症状。所有患者均无恶性肿瘤迹象。5例接受口服皮质类固醇治疗皮肤病的患者均有明显改善，未发生肌炎。这些病例进一步强调皮肌炎的皮肤表现是该病的典型病理表现，并挑战了通常认为肌肉疾病总是在皮肤病发病后2年内发生的观念。
We report six patients with the classic cutaneous findings of dermatomyositis who did not develop clinical or laboratory evidence of muscle disease for at least 2 years after onset of their skin manifestations. Such patients represent 11% of our total experience with dermatomyositis patients during a 15 year period. All six patients had Gottron's paules, periungual erythema/telangiectasia, and violaceous discoloration of the face, neck, upper chest, and back at some time during the course of their disease. In addition, all complained of pruritus and photosensitivity. None of the patients had evidence of malignancy. Each of five patients treated with oral corticosteroids for their cutaneous disease had marked improvement and did not develop myositis. These cases further emphasize that the cutaneous manifestations of dermatomyositis are pathognomonic of this disease and challenge the commonly held notion that muscle disease always develops within 2 years of onset of skin disease.