[2009] [THU0429] HIGH FREQUENCY ULTRASOUND (US) IN JUVENIL LOCALISED SCLERODERMA

F. Falcini, O. Kaloudi, F. Porta, F. Nacci, F. Bandinelli, S. Guiducci, M. Conforti, I. Miniati, M. Matucci Cerinic Department of Biomedicine, Division of Rheumatology, AOUC, University of Florence, Florence, Italy

Background: Juvenile Localized Scleroderma (JLS) causes functional disabilities and cosmetic deformities. Evaluation and follow up of cutaneous lesions is mandatory to understand the disease evolution.
Objectives: To evaluate the usefulness of US in assessing the characteristics of the skin lesions and monitoring the efficacy of drugs in JLS.
Methods: Ten patients (age: 101,7±66,2 months; 7 M, 3 F) affected by Juvenile onset LS (4 linear, 3 en coupe de sabre, 3 morphea) underwent sequential US. Skin thickness was measured with high frequency US (my Lab 25 Esaote®, 18 MHz probe). The measurement was made in the B mode image at the site where the demarcation lines among the epidermis, dermis, and subcutis were parallel. Seven patients started immunosuppressive therapy at baseline. All patients were re- evaluated both clinically (induration and extent of the lesions) and by US (dermal thickness and echogenicity) at 6 months. 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). 1/6 after 6 months was switched to Mycophenolate mofetil (25 mg/kg/day) due to inefficacy of MTX; 4/10 did not receive any therapy: 1 coupe de sabre due to relatives denial, 3 morphea due to limited extent of lesions.
Results: In all patients US detected increase of dermal echogenicity and loss of the subcutaneous fat that appeared disorganized and retracted at the level of the active lesions. Color and Doppler imaging, showed normal vessels density and size. After treatment, in 7 patients a clinical improvement, assessed as decrease of induration (from severe to moderate) and extent of lesions, was found. In 6 of these patients, US showed thinning and decrease of dermal echogenicity confirming the clinical improvement (significant correlation of clinical and US data with Fisher test p: 0.67). Three patients who did not receive drugs showed unmodified images and clinical findings.

Patient	Age at onset (months)	ANA pattern	Site of skin lesion	US at baseline	US at control	Clinical improvement	Therapy
1 MF	23	Negative	C. de sabre	2 mm	1.7 mm	Yes	Steroids MTX
2 VG	48	Negative	Thigh	2 mm	1.5 mm	Yes	None
3 PS	72	Negative	Thigh	1.4 mm	1.4 mm	No	None
4 DA	44	1:40	Lower extrem	1.6 mm	1.1 mm	Yes	Steroids MTX
5 PS	239	Negative	C. de sabre	3.4 mm	3.4 mm	Yes	Steroids MTX
6 SJR	72	Negative	C. de sabre	1.6 mm	1.6 mm	No	None
7 GAM	108	Negative	Multiples	1.1 mm	1 mm	Yes	MTX MMF
8 TS	179	1:320	Thigh	1.7 mm	1.5 mm	Yes	Steroids MTX
9 CS	120	Negative	Trunk, back	2.5 mm	2.5 mm	No	None
10 DR	112	Negative	Back	1.5 mm	1.3 mm	Yes	Steroids MTX

*MTX: methotrexate, **MMF: mycophenolate.

Conclusion: US seems to be a sensitive tool to evaluate skin modifications in JLS and correlate with clinical evaluation. Further studies are needed to better correlate clinical stage of lesions and morphological US images.
References:

1. Bendeck SE, Ultrasound as an outcome measure to assess disease activity in disorders of skin thickening: an example of the use of radiologic techniques to assess skin disease.Dermatologic therapy 2007.
2. Cosnes A et al. Thirteen-megahertz ultrasound probe:its role in diagnosing localized scleroderma. British J Dermatol 2003.
3. Li SC et al. Ultrasonography is a sensitive tool for monitoring localized scleroderma. Rheumatology 2007.