Mukamel M, Brik R
Rheumatology Unit, Schneider Children's Medical Center of Israel, Beilinson Campus, Petah Tiqva, Sackler Faculty of Medicine, Tel Aviv University, Israel.
J Clin Rheumatol. 2001 Jun;7(3):191-3. doi: 10.1097/00124743-200106000-00012.
Juvenile dermatomyositis is an inflammatory disease of unknown etiology that primarily affects skin and muscles. The pathognomonic Gottron's sign consists of symmetric macules and papules on the dorsal aspect of the interphalangeal joints and exterior areas of the big joints. A periorbital violaceous (heliotrope) skin rash is also characteristic. There may be a discordance in time of presentation of the skin and muscle disease, and a small subset of patients apparently do not develop muscle disease at all. The absence of muscle involvement is termed 'amyopathic dermatomyositis.'We describe two children who presented with the characteristic rash of juvenile dermatomyositis but with no clinical evidence of muscle involvement. One developed muscle weakness 3 years later. Neither patient had a full muscle work-up at the onset of the disease, which left questions about diagnoses and whether or not there may have been subtle muscle involvement. On the basis of our literature review, the outcome of these patients is uncertain, although it appears that myositis develops in many, maybe most, affected children. We suggest that in the absence of muscle disease, application of sunscreen and administration of hydroxychloroquine sulfate may ameliorate the rash. More aggressive treatment will need to be given when muscle involvement can be demonstrated.
幼年皮肌炎是一种病因不明的炎症性疾病，主要累及皮肤和肌肉。具有诊断意义的Gottron征表现为指间关节背侧及大关节外部区域的对称性斑疹和丘疹。眶周紫红色（向阳疹）皮疹也是其特征性表现。皮肤和肌肉疾病的出现时间可能不一致，一小部分患者显然根本不会发展为肌肉疾病。无肌肉受累被称为“无肌病性皮肌炎”。我们描述了两名儿童，他们出现了幼年皮肌炎的特征性皮疹，但无肌肉受累的临床证据。其中一名3年后出现肌肉无力。两名患者在疾病发作时均未进行全面的肌肉检查，这使得诊断以及是否可能存在轻微肌肉受累存在疑问。根据我们的文献综述，这些患者的预后尚不确定，尽管似乎许多（可能是大多数）受影响的儿童会发展为肌炎。我们建议，在无肌肉疾病的情况下，涂抹防晒霜和给予硫酸羟氯喹可能会改善皮疹。当证实有肌肉受累时，则需要进行更积极的治疗。
