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SP0206 Juvenile Localized Scleroderma: Assessing Disease Activity and Treatment
  1. F. Zulian
  1. Department of pediatrics, University of Padua, Padova, Italy

Abstract

During the recent years various methods for the clinical monitoring of localized scleroderma have been developed but none has been already validated in large cohort of patients.

Semi quantitative scoring methods, such as the Localized Scleroderma Severity Index (LoSSI), have been proposed. According to these scores the body surface is divided into several regions and skin thickness, inflammation and extension area are scored on a 0 – 3-point scale. These methods are quite subjective and do not evaluate the real size of the lesions. A computerized skin score (CSS) method for the measurement of circumscribed lesions in LS have been recently proposed. It consists in the demarcation of hyperemic and indurate borders of the scleroderma lesions on an adhesive transparent film, transfer over a cardboard, scanning and recording in a computer. Calculation of the affected area, performed by computer software, takes into account the child growth and allows, in this way, the longitudinal monitoring of the lesions.

Infrared thermography (IT) has been shown to be of value in the detection of active LS lesions in children with high sensitivity (92%) but low specificity (68%). The false positive results are related to the fact that old lesions lead to a marked atrophy of skin, subcutaneous fat and muscle, with increased heat conduction from deeper tissues.

Laser doppler flowmetry (LDF) is a non-invasive method for the measurement of cutaneous microcirculation. Blood flow, measured by LDF, is significantly increased in clinically active lesions with high sensitivity and specificity. LDF and IT can be complementary tools in evaluating LS: IT may reveals active hidden lesions while LDF is useful to exclude activity in atrophic lesions, falsely positive with IT.

Skin imaging with high-frequency ultrasound (USG) has been found to be useful in monitoring therapy in a preliminary study. Ultrasonography can detect several abnormalities such as increased blood flow, increased echogenicity due to fibrosis and loss of subcutaneous fat. The main limits of this tool are its operator-dependency and the lack of standardization.

Magnetic Resonance Imaging (MRI) is indicated in the linear subtype when CNS or orbital involvement are suspected. In the other forms, involving the limbs, MRI is able to demonstrate the extent and depth of soft tissue lesions. The two main disadvantages are the need for sedation in younger patients and the presence of possible artifacts.

As for treatment, no therapy is usually needed for the single circumscribed lesion, as in circumscribed morphea, but emollients (any lanolin-based cream) and low concentration topical corticosteroids may help relieve dryness and itching.

Systemic treatment is recommended when there is a significant risk for disability, such as in deep pansclerotic morphea, progressive linear morphea crossing joint lines or involving the face (en coup de sabre), or generalized morphea.

Methotrexate has been used successfully in children and adults with LSc. We recommend a weekly regimen of methotrexate of 15 mg/m2, given as a single oral or subcutaneous dose per week for at least one year. During the first two to three months of therapy, a course of glucocorticoids may be used as adjunctive bridge therapy for those with rapidly progressive disease. Patients who do not respond to this treatment approach may be treated with mycophenolate mofetil at a dose of 500-1000 mg/m2. The use of ultraviolet light therapy, with or without chemical agents such as psoralen, has been reported to be beneficial for localized or superficial lesions in a number of studies.

Disclosure of Interest None declared

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