Porta Francesco, Kaloudi Olga, Garzitto Alice, Prignano Francesca, Nacci Francesca, Falcini Fernanda, Matucci Cerinic Marco
Department of Sperimental and Clinical Medicine, Section Rheumatology, AOUC, University of Florence , Florence , Italy.
Mod Rheumatol. 2014 Sep;24(5):869-73. doi: 10.3109/14397595.2013.844301. Epub 2013 Oct 18.
Juvenile Localized Scleroderma (JLS) causes functional disabilities and cosmetic deformities. Evaluation and follow-up of lesions are mandatory to understand the disease evolution. The objective of this study is to evaluate the usefulness of skin ultrasonography (US) in monitoring the response to treatment in JLS.
Ten patients (age: 101,7 ± 66,2 months; 7 M, 3 F) affected by juvenile onset LS underwent sequential US exams (at baseline and after 6 months). Skin thickness was measured by using high-frequency US (18 MHz). All patients were evaluated both clinically (modified Rodnan Skin Score, mRSS) and by US (dermal thickness) at baseline and at 6 months. At baseline, 6/10 patients received 3 pulses of corticosteroids (solumedrol 30 mg/kg/day for 3 consecutive days, then oral steroids (1mg/kg), and methotrexate s.c. (15 mg/mq/week). After 6 months, 1/6 was switched to mycophenolate mofetil (25 mg/kg/day) due to inefficacy of MTX; 4/10 did not receive any further therapy.
US showed a thicker dermis and a thinned hypodermis in the lesional skin areas in respect to the healthy ones (p < 0.05). After treatment, in seven patients a clinical improvement (decrease of mRSS) was found. In six of these patients, US showed a decrease of dermal thickness showing a correlation with clinical data. Three patients who did not receive drugs showed unmodified images and clinical findings.
US can help the assessment of skin and hypodermis in JLS and can detect an improvement of the lesions.
青少年局限性硬皮病（JLS）会导致功能残疾和外观畸形。对病变进行评估和随访对于了解疾病进展至关重要。本研究的目的是评估皮肤超声检查（US）在监测JLS治疗反应中的实用性。
10例青少年起病的局限性硬皮病患者（年龄：101.7±66.2个月；男7例，女3例）接受了连续的超声检查（基线时和6个月后）。使用高频超声（18MHz）测量皮肤厚度。所有患者在基线时和6个月时均接受临床评估（改良Rodnan皮肤评分，mRSS）和超声检查（真皮厚度）。基线时，6/10的患者接受了3次皮质类固醇脉冲治疗（甲强龙30mg/kg/天，连续3天，然后口服类固醇（1mg/kg），以及皮下注射甲氨蝶呤（15mg/m²/周）。6个月后，1/6的患者因甲氨蝶呤无效而改用霉酚酸酯（25mg/kg/天）；4/10的患者未接受任何进一步治疗。
与健康皮肤相比，超声显示病变皮肤区域的真皮更厚，皮下组织更薄（p<0.05）。治疗后，7例患者出现临床改善（mRSS降低）。其中6例患者的超声显示真皮厚度降低，与临床数据相关。3例未接受药物治疗的患者图像和临床发现未改变。
超声有助于评估JLS患者的皮肤和皮下组织，并能检测病变的改善情况。
