Virdi Annalucia, Patrizi Annalisa, Cambiaghi Stefano, Diociaiuti Andrea, El Hachem May, Schena Donatella, Bassi Andrea, Bonamonte Domenico, Brazzelli Valeria, Belloni Fortina Anna, Pepe Patrizia, DI Lernia Vito, Neri Iria
Division of Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy -
Division of Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.
Ital J Dermatol Venerol. 2021 Aug;156(4):446-454. doi: 10.23736/S2784-8671.20.06632-8. Epub 2020 Oct 16.
There are still few dermatological studies on morphea. We evaluated the epidemiological and clinical features and management of pediatric morphea, reporting dermatologists experience.
A multicenter retrospective observational study was carried out on the epidemiological and clinical features and management of the disease between 01/01/2009 and 01/10/2014 in 10 Italian Dermatological Units.
We collected the data of 69 children affected by: circumscribed morphea (39.1%); linear morphea of trunk and limbs (14.5%); en coupe de sabre morphea (ECDS) (14.5%); progressive facial hemiatrophy (8.7%); generalized form (18.8%); mixed morphea (4.4%). The mean age at onset was 6.86±3.21 years, mainly between 2 and 8 years, but is statistically significantly lower for ECDS (4.5±3.03). Localizations were: head/neck (30.4%), limbs (26.1%), trunk (14.5%), 2 or more sites (29%), most often the trunk plus limbs. Extracutaneous manifestations were observed in 26.1% patients. 10 patients presented a second autoimmune disorder. Treatments were topical in 26.1% cases and systemic (alone or associated with topical treatments) in 68.1%.
There was a lack of uniformity in the management of patients and an increasing awareness of dermatologists on the use of systemic therapies, in particular of methotrexate, which is no longer exclusive to rheumatologists. Methotrexate causes stabilization and improvement of the clinical signs, but topical creams are still considered adjuvant or maintenance therapies during/after the use of systemic drugs.
关于硬斑病的皮肤病学研究仍然较少。我们评估了儿童硬斑病的流行病学、临床特征及治疗情况，并报告皮肤科医生的经验。
2009年1月1日至2014年10月1日期间，在意大利的10个皮肤科单位对该病的流行病学、临床特征及治疗情况进行了一项多中心回顾性观察研究。
我们收集了69例儿童患者的数据，其类型包括：局限性硬斑病（39.1%）；躯干和四肢线状硬斑病（14.5%）；剑劈状硬斑病（ECDS）（14.5%）；进行性面部半侧萎缩（8.7%）；泛发型（18.8%）；混合型硬斑病（4.4%）。发病的平均年龄为6.86±3.21岁，主要在2至8岁之间，但ECDS的发病年龄在统计学上显著更低（4.5±3.03岁）。病变部位包括：头颈部（30.4%）、四肢（26.1%）、躯干（14.5%）、两个或更多部位（29%），最常见的是躯干加四肢。26.1%的患者出现了皮肤外表现。10例患者出现了第二种自身免疫性疾病。26.1%的病例采用局部治疗，68.1%采用全身治疗（单独或与局部治疗联合）。
患者的治疗缺乏一致性，皮肤科医生对全身治疗尤其是甲氨蝶呤的使用认识不断提高，甲氨蝶呤不再是风湿病学家的专属用药。甲氨蝶呤可使临床症状稳定并改善，但局部乳膏在使用全身药物期间/之后仍被视为辅助或维持治疗。
