Purpose or Case Report: Juvenile localized scleroderma (JLS) is a rare chronic autoimmune connective tissue disorder characterized by inflammation and fibrosis of the skin and underlying subcutaneous tissues. It may affect the trunk, extremities or, less frequently, the face and head. Early diagnosis and close monitoring are essential to prevent functional impairment and cosmetic sequelae. However, clinical examination alone may underestimate disease activity, particularly in deep or subtle lesions. Aim of this study is to highlight the role of high-frequency Ultrasound (US) in detecting and monitoring JLS, emphasizing its value in assessing disease activity, depth of involvement, and response to treatment.
Methods & Materials: We present eight pediatric JLS cases (one boy and seven girls, aged 3 –14 years) retrieved from our Radiology Department database. US examinations, both at diagnosis and during a 1-8 year follow-up, were performed with high-frequency (10-24 MHz) linear probes, and were supplemented with color and power Doppler, and microvascular imaging (MVI) techniques.
Results: The spectrum of sonographic JLS findings in various anatomic locations (including face, salivary glands, tongue, trunk, and extremities) and throughout all JLS stages is presented. A protocol of the US examination in different body regions is proposed. Active, inactive and atrophic lesions were differentiated based on dermal and subcutaneous thickness and echogenicity, definition of the dermal-hypodermal border, and degree of vascularity within the dermis and subcutaneous tissue. High-frequency gray-scale US, combined with color Doppler and MVI, enabled precise measurement of dermal and subcutaneous thickness, identification of subtle dermal and subcutaneous changes, and monitoring of lesion evolution. Notably, US also detected subclinical disease activity, allowing timely treatment adjustments.
Conclusions: High-frequency gray-scale US, supplemented with Doppler and MVI, is an indispensable imaging tool in the evaluation of JLS. It enhances the detection of active disease, quantifies tissue involvement and vascularity, and allows reliable follow-up of disease progression and treatment response. A standardized US protocol and awareness of the characteristic US patterns of JLS are key for pediatric radiologists to optimize disease assessment, staging, and monitoring of disease evolution.