Section on Nervous System Development and Plasticity, The Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, MD 20892, USA. Electronic address: fieldsd@mail.nih.gov.
Centre for Regenerative Medicine, Institute for Regeneration and Repair, University of Edinburgh, Edinburgh EH16 4UU, United Kingdom M.R.Livesey@Sheffield.ac.uk m.swire@ucl.ac.uk.
Centre for Discovery Brain Sciences, University of Edinburgh, Edinburgh EH8 9XD, United Kingdom.
Wolfson Institute for Biomedical Research, University College London, London WC1E 6BT, United Kingdom.
The role and potential molecular mechanism of inflammatory cells in pediatric localized scleroderma are poorly investigated. In this study, we first investigated the profiling of inflammatory cells in...
Connective tissue nevi (CTN) are congenital hamartomas caused by excessive proliferation of dermis components. In children, CTN can mimic juvenile localized scleroderma (JLS), an immune mediated skin ...
Aim of our study was to describe a series of pediatric patients with CTN misdiagnosed as JLS and the discerning characteristics between the two conditions....
Retrospective analysis of children referred to our Center during the last two decades for JLS who received a final diagnosis of CTN. Clinical, laboratory, histopathological and instrumental data (MRI ...
Seventeen patients with mean age at onset 4.6 years entered the study. All came to our Center with a certain diagnosis of JLS (n = 15) or suspected JLS (n = 2). The indurated skin lesions were flat an...
CTN can be misdiagnosed as JLS and therefore aggressively treated with prolonged and inappropriate immunosuppression. The absence of inflammatory appearance of the skin lesions, normal instrumental an...
Juvenile localized scleroderma (JLS) or morphoea, a rare chronic autoimmune disease predominantly affects skin, subcutaneous tissue and occasionally the adjacent muscle, fascia and bone. We report the...
Patients who were diagnosed to have JLS were enrolled from the Paediatric Dermatology Clinic and the Paediatric Rheumatology Clinic of a tertiary care referral hospital in India. Collected data includ...
We analysed 84 patients with Juvenile localized scleroderma. Median age of disease onset was 5 years, and median age at diagnosis was 8 years. Commonest subtype was linear scleroderma (57 patients, 67...
Early use of systemic corticosteroids along with methotrexate may be more beneficial than methotrexate therapy alone....
Current treatment for localized scleroderma (LS) has been shown to halt disease activity, but little is still known about patient experiences with these treatments, nor is there consensus about optima...
Conduct a scoping review of the literature for the types of outcomes and measures (i.e. clinician-, patient-, and caregiver-reported) utilized in published treatment studies of LS....
Online databases were searched for articles related to the evaluation of treatment efficacy in LS with a special focus on pediatrics....
Of the 168 studies, the most common outcomes used were cutaneous disease activity and damage measured via clinician-reported assessments. The most frequently cited measure was the Localized Scleroderm...
Some studies only vaguely reported the measures utilized, and the review yielded a low number of clinical trials....
In addition to evaluating disease activity with clinician-reported measures, the field could obtain critical knowledge on the patient experience by including high-quality PROMs of symptoms and functio...
Infrared thermography (IRT) is a useful method to detect activity/inflammation in localized scleroderma (LoS); however, inactive skin lesions with a severe degree of dermal and subcutaneous atrophy ma...
The aim of this research was to compare the spectrophotometric results with thermographic examination of LoS lesions....
The lesions were assessed using the Localized Scleroderma Assessment Tool (LoSCAT), Dyspigmentation, Induration, Erythema, and Telangiectasias (DIET) score, NBRS and IRT. The difference in the erythem...
Fifty-five patients with 49 active and 64 inactive LoS lesions were examined. The ΔEI strongly correlated with the erythema (r...
Narrow-band reflectance spectrophotometry may be a complementary method for determining erythema in LoS active lesions, although this technique remains inferior to IRT, because is unable to distinct b...
Localized scleroderma (LS) is an autoimmune disease with both inflammatory and fibrotic components causing an abnormal deposition of collagen in the skin and underlying tissue, often leading to disfig...
Localized scleroderma (LS) is characterized by skin fibrosis, hyperpigmentation and soft tissue atrophy. Fat grafting has been widely used to correct LS deformity....
To investigate the effect of fat grafting on the skin pigmentation of LS lesions....
A prospective self-controlled study was conducted. Skin melanin and erythema indexes were measured by Mexameter® MX18 before and 3 months after surgery. Differences between lesions and contralateral n...
Fourteen frontal linear LS patients participated in the study. Before surgery, the melanin index of the lesions was significantly higher than the contralateral sites (p = 0.023), while the erythema in...
Fat grafting could alleviate skin hyperpigmentation and skin damage of LS lesions while having little effect on skin erythema and disease activity....
This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Inst...
Scleroderma is a rare complication in taxanes therapy. Although individual cases of taxanes-induced scleroderma have been reported, the clinical manifestation and treatment outcomes were reviewed and ...
A PubMed literature review on published cases of taxanes-induced scleroderma up until April 2022 was included for analysis....
The search identified 27 patients with adequate information for analysis. Of the 28 patients, including the one presented here, 22 were female. Peripheral edema was the most common symptom in all but ...
Taxanes-induced scleroderma is different from idiopathic scleroderma. Physicians should be aware of this condition in order to provide early diagnosis and apply appropriate management in order to avoi...
Localized scleroderma (LS) is an autoimmune disease characterized by inflammation and fibrosis, leading to severe cutaneous manifestations such as skin hardening, tightness, discoloration, and other t...
To support physical activity among people with systemic sclerosis (SSc [scleroderma]), we sought to determine the prevalence and importance of barriers and the likelihood of using possible facilitator...
We invited 1,707 participants from an international SSc cohort to rate the importance of 20 barriers (14 medical, 4 social or personal, 1 lifestyle, and 1 environmental) and the likelihood of using 91...
Among 721 respondents, 13 barriers were experienced by ≥25% of participants, including 2 barriers (fatigue and Raynaud's phenomenon) rated "important" or "very important" by ≥50% of participants, 7 ba...
Medical-related physical activity barriers were common and considered important. Facilitators considered as most likely to be used involved adapting exercise, taking care of one's body, keeping warm, ...
