Division of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Division of Rheumatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Indian J Dermatol Venereol Leprol. 2022 Mar-Apr;88(2):162-170. doi: 10.25259/IJDVL_297_20.
Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients.
To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children.
This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019.
Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron's rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset.
This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study.
juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur.
幼年型皮肌炎是一种罕见疾病，但它是儿科患者中最常见的特发性炎性肌病。
研究泰国儿童幼年型皮肌炎的临床表现、检查、治疗、临床病程和结局。
这项回顾性研究纳入了 1994 年至 2019 年在泰国曼谷的 2300 张床位的国家三级转诊中心诗里拉吉医院接受治疗的幼年型皮肌炎患者。
30 名患者（22 名女性和 8 名男性）入选，女性与男性的比例为 2.7:1。诊断时的中位年龄为 5.1 岁（范围，2.6-14.8 岁）。中位确诊前病程为 6.5 个月（范围，0.3-84.0 个月）。大多数患者为急性或亚急性起病。27/30（90%）例患者出现肌肉无力，26/30（86.7%）例患者出现皮疹，17/26（65.4%）例患者出现肌肉疼痛，4/18（22.2%）例患者出现关节痛。皮肤科检查发现 25/30（83.3%）例患者有 Gottron 征皮疹，21/30（70.0%）例患者有向阳疹，15/24（62.5%）例患者有甲周毛细血管扩张。有趣的是，21 例患者中有 8 例（38.1%）出现头皮炎。本系列中最常用的治疗方案是泼尼松龙联合甲氨蝶呤。中位随访 3.1 年（范围，0.0-18.5 年）期间，仅三分之一的患者被认为是单环疾病。1 例患者在幼年型皮肌炎发病 12 年后于先前钙沉积性皮病的部位发生骨肉瘤。
这是一项回顾性单中心研究，我们的结果可能不适用于其他医疗环境。需要前瞻性多中心研究来证实本研究的结果。
幼年型皮肌炎通常具有诊断和治疗方面的挑战，而在临床表现方面存在种族差异，这在本研究中得到了证实。亚洲患者往往表现为疾病的急性或亚急性发作，关节痛和/或关节炎比白人患者少见。头皮炎在儿科幼年型皮肌炎患者中并不少见。幼年型皮肌炎与恶性肿瘤的关联虽然罕见，但也可能发生。
