Trautmann C, Abdel-Naser M B, Soehnchen R, Detmar M, Orfanos C E
Universitäts-Hautklinik und Poliklinik, Freien Universität, Berlin.
Hautarzt. 1995 Jan;46(1):47-52. doi: 10.1007/s001050050207.
The so-called amyopathic dermatomyositis is a rare variant of dermatomyositis which has attracted increasing interest during the last years. One finds the classical signs of dermatomyositis such as periorbital edema and erythema, erythematous macular and papular lesions localized at bony prominences (so-called Gottron's papules), generalized pruritus, photosensitivity, and a cutaneous histopathologic picture compatible with skin lesions of dermatomyositis. Crucial for the diagnosis is the exclusion of myositis by clinical examination, EMG and histology. Furthermore, longterm supervision of patients is advisable in order not to miss the appearance of early signs of myositis. The longest reported follow-up of amyopathic dermatomyositis patient is 4 years; however, it cannot be excluded that these cases will eventually culminate in classical dermatomyositis. In this paper we describe two cases and discuss the differential diagnosis and therapy; also, the term "Premyopathic dermatomyositis" is proposed, to indicate that the full picture is to be expected in most cases.
所谓无肌病性皮肌炎是皮肌炎的一种罕见变异型，在过去几年中受到越来越多的关注。人们会发现皮肌炎的典型体征，如眶周水肿和红斑、位于骨隆突处的红斑性斑疹和丘疹性皮损（所谓的Gottron丘疹）、全身性瘙痒、光敏性以及与皮肌炎皮肤病变相符的皮肤组织病理学表现。诊断的关键在于通过临床检查、肌电图和组织学排除肌炎。此外，建议对患者进行长期监测，以免错过肌炎早期体征的出现。报道的无肌病性皮肌炎患者最长随访时间为4年；然而，不能排除这些病例最终会发展为典型皮肌炎。在本文中，我们描述了两例病例并讨论了鉴别诊断和治疗；此外，还提出了“肌炎前期皮肌炎”这一术语，以表明在大多数情况下可能会出现完整的症状表现。
