Global research status of localised scleroderma reported over the period 1993-2022: A 30-year bibliometric analysis.

Localised scleroderma predominantly affects the skin with an unknown aetiology. Despite its clinical importance, no comprehensive bibliometric analysis has been conducted to assess the existing research landscape and future prospects for localised scleroderma. The articles related to localised scleroderma were retrieved from the WoSCC database and analysed by VOSviewer 1.6.10.0 (Leiden University, Netherlands), CiteSpace 6.1.R1 (Dreiser University, USA), and HistCite 2.1 (New York, United States). 2049 research papers pertaining to localised scleroderma spanning the years from 1993 to 2022 were extracted from the WoSCC database. The United States exhibited the highest productivity with 644 papers, accounting for 31.43% of the total output, followed by Germany with 206 papers (10.05%) and Italy with 200 papers (9.76%). Regarding academic institutions and journals, the University of Texas System and Dermatology published the most significant number of papers, and Professor Ihn, H emerged as the most prolific contributor among scholars. The top 10 cited references primarily concentrated on the diagnosis and treatment of localised scleroderma. "Phototherapy" and "methotrexate (MTX)" surfaced as the most recent and noteworthy keywords, representing the research hotspots in the domain of localised scleroderma. This bibliometric analysis furnishes valuable insights into the contemporary research landscape of localised scleroderma.

Utility of phototherapy in patients with systemic sclerosis: Systematic review.

Phototherapy is a recommended treatment regimen for different scleroderma spectrum disorders, but so far it has been included neither by European nor by worldwide experts committee in recommendations for the treatment of systemic sclerosis (SSc). The aim of the study was to revisit the utility of dermatological phototherapy in patients with SSc. PubMed using medical subject headings was searched to identify studies evaluating response to dermatological phototherapy in SSc patients. Both UVA1 (340-400 nm) and PUVA (psoralen plus UVA) treatments were found to reduce skin thickening and increase skin elasticity, therefore allowing for the improvement of joint tension mobility, especially in hands. At least several papers showed efficacy of phototherapy in patients who remained non-responsive to previous immunosuppressive therapies. The most probable mechanisms of action of phototherapy in SSc include inhibition of T-cells and prevention from dermal fibrosis. Although most data on the efficacy of phototherapy come from small experimental studies and case reports, phototherapy based on UVA of wavelength manifests relatively mild spectrum of side effects and this should be considered as a treatment option for SSc with dominant cutaneous involvement.

UVA-1 phototherapy as adjuvant treatment for eosinophilic fasciitis: in vitro and in vivo functional characterization.

INTRODUCTION: Eosinophilic fasciitis (EF) is a rare autoimmune disease causing progressive induration of dermal, hypodermal, and muscularis fascia. The exact pathogenesis is yet to be fully understood, and a validated therapy protocol still lacks. We here aimed to realize a clinical-functional characterization of these patients. MATERIALS AND METHODS: A total of eight patients (five males, 45 years average) were treated with adjuvant high-dose UVA-1 phototherapy (90 J/cm), after having received the standard systemic immunosuppressive protocol (oral methylprednisolone switched to methotrexate). Body lesion mapping, Localized Scleroderma Assessment Tool (LoSCAT), Dermatology Life Quality Index (DLQI), High-Resolution Ultrasound (HRUS) (13-17MHz), and ultra HRUS (55-70 MHz) were performed at each examination time taking specific anatomical points. Gene expression analysis at a molecular level and in vitro UVA-1 irradiation was realized on lesional fibroblasts primary cultures. RESULTS: The LoSCAT and the DLQI showed to decrease significantly starting from the last UVA-1 session. A significant reduction in muscularis fascia thickness (-50% on average) was estimated starting from 3 months after the last UVA-1 session and maintained up to 12 months follow-up. Tissues was detected by HRUS. The UVA-1 in vitro irradiation of lesional skin sites cells appeared not to affect their viability. Molecular genes analysis revealed a significant reduction of IL-1ß and of TGF-ß genes after phototherapy, while MMPs 1,2,9 gene expression was enhanced. COMMENT: These preliminary in vivo and in vitro findings suggest that UVA-1 phototherapy is a safe and useful adjuvant therapy able to elicit anti-inflammatory effects and stimulate tissue matrix digestion and remodeling at lesional sites.

Morfea superficial / Superficial morphea

La morfea superficial es una variante rara de morfea que se distingue de la clásica tanto en la clínica como en la histopatología. Se caracteriza por máculas hipopigmentadas o hiperpigmentadas, con mínima o ninguna induración, sin síntomas asociados, contractura ni atrofia. En la histopatología, se observa un compromiso limitado a las fibras colágenas en la dermis reticular superficial. Se comunica el caso de una paciente con diagnóstico de morfea superficial tratada con fototerapia ultravioleta B y metotrexato.

Superficial morphea is a rare variant of morphea that is distinguished from the classic variant both clinically and histopathologically. It is characterized by hypo or hyperpigmented patches with minimal to no induration, without associated symptoms, without contracture or atrophy. At the histopathological level, a limited involvement of collagen fibers is observed at the level of the uperficial reticular dermis. The case of a patient with superficial morphea treated with ultraviolet B phototherapy and methotrexate is presented.

Comparative efficacy and safety of traditional Chinese medicine for lipodermatosclerosis: A protocol for systematic review and network meta-analysis.

BACKGROUND: Lipodermatosclerosis (LDS) is a severe skin change accompanied by the development of chronic venous disease of the lower extremities. Its main clinical manifestations are erythema, induration, hyperpigmentation, and rough and thickened skin. It may also eventually lead to refractory ulcers, skin necrosis and even cancer. Conventional treatment methods mainly include the intake of oral anabolic hormones or androgen and pressure therapy. However, patients often refuse due to their drug resistance and intolerance. As a clinical irreplaceable treatment method for LDS, traditional Chinese medicine (TCM) has not been compared of the safety and effectiveness so far. Therefore, we cannot wait to use a method to compare the efficacy of TCM for LDS systematically, such as network meta-analysis (NMA). METHODS: We will retrieve the relevant Chinese and English databases comprehensively. All the randomized controlled trials of TCM for LDS from January 2015 to September 2020 will be included. Under the guidance of inclusion criteria, 2 researchers will screen the literature, then assess the risk of bias and extract data. We will use Bayesian NMA to evaluate all available evidence in STATA 14.0 and WinBUGS software. RESULTS: This study will use Bayesian NMA to evaluate the efficacy and safety of TCM for LDS. CONCLUSION: This study provide a reliable theoretical basis for the clinical application of TCM in the treatment of LDS, and contribute to the formulation of treatment guidelines for LDS.

Phototherapy in sclerosing and pseudo-sclerosing skin diseases Fototerapia en enfermedades cutáneas esclerosantes y pseudoesclerosantes.

Sclerosing and pseudo-sclerosing skin diseases are a therapeutic challenge. Ultraviolet radiation, depending on its wavelength, penetrates into different layers of the skin and acts on cells that promote tissue remodeling and differentiation, such as keratinocytes and fibroblasts. Furthermore, it modulates the inflammatory processes in dendritic cells, endothelial cells, and leukocytes by intervening in the production of cytokines and profibrotic molecules. For these reasons ultraviolet light is a useful option in the treatment of these conditions. Las enfermedades esclerosantes y pseudoesclerosantes de la piel son un grupo de dermatosis que suponen un reto terapéutico para el clínico. La radiación ultravioleta, de acuerdo con su longitud de onda, penetra en las diferentes capas de la piel y actúa sobre aquellas células que favorecen la diferenciación y remodelación tisular como queratinocitos y fibroblastos. Además, modula los procesos inflamatorios en células dendríticas, endoteliales y leucocitos al intervenir en la producción de citoquinas y moléculas profibróticas, volviéndose una alternativa útil en el tratamiento de estas condiciones.

Beneficial Effects of Near-Infrared Light Photobiomodulation in Linear Morphea: A Case Report.

Background: Linear morphea is a variant of scleroderma limited to the skin and underlying tissues secondary to an autoimmune inflammation leading to excess collagen deposition and fibrosis. Apart from topical or oral medications, successful light-based treatments have been reported using phototherapy including Psoralen plus ultraviolet A, photodynamic therapy, carbon dioxide laser, pulsed dye laser, and visible/infrared light. Methods: We report a patient with biopsy-proven infraorbital linear morphea responding to 940 nm near-infrared light photobiomodulation treatments. Results: The patient had excellent cosmesis without textural changes or hypopigmentation despite her darker skin complexion (Fitzpatrick phototype III) after tri-weekly treatments for 8 months. Conclusions: Linear morphea, therefore, may be potentially amenable to home use light-based therapy by using nonthermal nonablative 940 nm photons. To our knowledge, this home-based treatment approach has not been previously reported.

Paediatric morphoea: a holistic review. Part 2: diagnosis, measures of disease activity, management and natural history.

Paediatric morphoea is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Defining optimum management strategies in paediatric morphoea remains an ongoing challenge, owing to the varied presentations and a relative paucity of paediatric-specific studies. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected and results analysed before summarizing them. In Part 1 of this review, we described the epidemiology, aetiopathogenesis and clinical classification; in this part, we discuss the diagnosis, markers of disease activity, management and natural history in paediatric morphoea.

Long-standing morphea and the risk of squamous cell carcinoma of the skin.

Scleroderma is a heterogeneous group of fibrosing connective tissue disorders of unknown etiology. Morphea is a localized form of scleroderma that occasionally leads to chronic erosions and ulcerations of the skin. Fibrosis, inflammation and chronic ulcerations may eventually promote skin neoplasms; morphea is therefore a rare but established risk factor for cutaneous squamous cell carcinoma (cSCC). We present a review of 16 scleroderma patients: 15 case reports from the literature (identified by a PubMed search) and one case from our clinic of a patient who had developed cSCC, and we discuss potential underlying mechanisms. Statistical analysis revealed that the lower extremities were the body site most commonly affected by cSCC in these scleroderma patients. The mean time interval between the onset of scleroderma and the development of cSCC was ten to twenty years. In patients with morphea, we recommend checking for skin tumors during follow-up examinations as well as a careful risk-benefit analysis when considering the application of immunosuppressants or phototherapy in view of their potential carcinogenic side effects.

Overview of Juvenile localized scleroderma and its management.

BACKGROUND: Juvenile localized scleroderma (JLS) is a rare pediatric disease characterized by inflammation and skin thickening. JLS is associated with deep tissue and extracutaneous involvement that often results in functional impairment and growth disturbances. This article provides an overview of the disease with a focus on active features and treatment. DATA SOURCES: We searched databases including PubMed, Elsevier and MedLine and Wanfang, reviewing publications from 2013 to 2019. Selected earlier publications were also reviewed. RESULTS: Linear scleroderma is the most common JLS subtype. Several lines of evidence suggest that JLS is an autoimmune disease. Extracutaneous involvement is common and can present before the onset of skin disease. Multiple skin features are associated with disease activity, and activity can also manifest as arthritis, myositis, uveitis, seizures, and growth impairment. Systemic immunosuppressive treatment, commonly methotrexate with or without glucocorticoids, greatly improves outcome and is recommended for treating JLS patients with active disease and moderate or higher severity. Long term monitoring is needed because of the disease's chronicity and the high frequency of relapses off of treatment. CONCLUSIONS: JLS is associated with a risk for disabling and disfiguring morbidity for the growing child. Identifying active disease is important for guiding treatment, but often difficult because of the paucity of markers and lack of a universal skin activity feature. More studies of JLS pathophysiology are needed to allow the identification of biomarkers and therapeutic targets. Comparative effectiveness treatment studies are also needed to work towards optimizing care and outcome.

Morphea: a practical review of its diagnosis, classification and treatment

Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.

Interventions for morphea.

BACKGROUND: Morphea (morphoea) is an immune-mediated disease in which excess synthesis and deposition of collagen in the skin and underlying connective tissues results in hardened cutaneous areas. Morphea has different clinical features according to the subtype and stage of evolution of the disease. There is currently no consensus on optimal interventions for morphea. OBJECTIVES: To assess the effects of treatments for people with any form of morphea. SEARCH METHODS: We searched the following databases up to July 2018: the Cochrane Skin Specialised Register, CENTRAL, MEDLINE, Embase, LILACS, and five trial registers. We checked the reference lists of included studies for further references to relevant randomised controlled trials. SELECTION CRITERIA: Randomised controlled trials of topical, intralesional, or systemic treatments (isolated or combined) in anyone who has been clinically diagnosed by a medical practitioner with any form of morphea. Eligible controls were placebo, no intervention, any other treatment, or different doses or duration of a treatment. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. The primary outcomes were global improvement of disease activity or damage assessed by a medical practitioner or by participants, and adverse effects. Secondary outcomes were improvement of disease activity and improvement of disease damage. We used GRADE to assess the quality of the evidence for each outcome. MAIN RESULTS: We included 14 trials, with a total of 429 randomised participants, aged between 3 and 76 years. There were juvenile and adult participants; over half were female, and the majority had circumscribed morphea, followed by linear scleroderma. The settings of the studies (where described) included a dermatologic centre, a national laboratory centre, paediatric rheumatology and dermatology centres, and a university hospital or medical centre.The studies evaluated heterogenous therapies for different types of morphea, covering a wide range of comparisons. We were unable to conduct any meta-analyses. Seven studies investigated topical medications, two evaluated intralesional medications, and five investigated systemic medications. The study duration ranged from seven weeks to 15 months from baseline.We present here results for our primary outcomes for our four key comparisons. All of these results are based on low-quality evidence.The included studies were at high risk of performance, detection, attrition, and reporting bias.Global improvement of disease activity or damage after treatment may be higher with oral methotrexate (15 mg/m², maximum 20 mg, once a week, for 12 months or until disease flare) plus oral prednisone (1 mg/kg a day, maximum of 50 mg, in a single morning dose, for three months, and one month with gradually decreased dose until discontinuation) than with placebo plus oral prednisone in children and adolescents with active morphea (linear scleroderma, generalised morphea or mixed morphea: linear and circumscribed) (risk ratio (RR) 2.31, 95% confidence interval (CI) 1.20 to 4.45; number needed to treat for an additional beneficial outcome (NNTB) 3; 1 randomised controlled trial (RCT); 70 participants, all juvenile). This outcome was measured 12 months from the start of treatment or until flare of the disease. Data were not available separately for each morphea type. There may be little or no difference in the number of participants experiencing at least one adverse event with oral methotrexate (26/46) or placebo (11/24) (RR 1.23, 95% CI 0.75 to 2.04; 1 RCT; 70 participants assessed during the 12-month follow-up). Adverse events related to methotrexate included alopecia, nausea, headache, fatigue and hepatotoxicity, whilst adverse events related to prednisone (given in both groups) included weight gain (more than 5% of body weight) and striae rubrae.One three-armed RCT compared the following treatments: medium-dose (50 J/cm²) UVA-1; low-dose (20 J/cm²) UVA-1; and narrowband UVB phototherapy. There may be little or no difference between treatments in global improvement of disease activity or damage, as assessed through the modified skin score (where high values represent a worse outcome): medium-dose UVA-1 phototherapy versus low-dose UVA-1 group: MD 1.60, 95% CI -1.70 to 4.90 (44 participants); narrowband UVB phototherapy versus medium-dose UVA-1 group: MD -1.70, 95% CI -5.27 to 1.87 (35 participants); and narrowband UVB versus low-dose UVA-1 group: MD -0.10, 95% CI -2.49 to 2.29 (45 participants). This RCT included children and adults with active morphea (circumscribed morphea, linear scleroderma (with trunk/limb variant and head variant), generalised morphea, or mixed morphea), who received phototherapy five times a week, for eight weeks. Outcomes were measured at eight weeks from the start of treatment.Safety data, measured throughout treatment, from the same RCT (62 participants) showed that treatment with UVA-1 phototherapy may cause mild tanning compared to narrowband UVB: narrowband UVB versus medium-dose UVA-1: RR 0.03, 95% CI 0.00 to 0.42; 35 participants; narrowband UVB versus low-dose UVA-1: RR 0.03, 95% CI 0.00 to 0.41; 45 participants. However, there may be no difference in the number of participants reporting mild tanning when comparing medium and low dose UVA-1 phototherapy (RR 1.00, 95% CI 0.91 to 1.10; 44 participants). Transient erythema was reported in three participants with narrowband UVB and no participants in the low- or medium-dose UVA-1 groups. AUTHORS' CONCLUSIONS: Compared to placebo plus oral prednisone, oral methotrexate plus oral prednisone may improve disease activity or damage in juvenile active morphea (linear scleroderma, generalised morphea or mixed morphea: linear and circumscribed), but there may be a slightly increased chance of experiencing at least one adverse event.When medium-dose UVA-1 (50 J/cm²), low-dose UVA-1 (20 J/cm²), and narrowband UVB were compared against each other in treating children and adults with active morphea (circumscribed morphea, linear scleroderma, generalised morphea and mixed morphea), there may be little or no difference between these treatments on global improvement of disease activity or damage. UVA-1 phototherapy may cause more mild tanning than narrowband UVB, but there may be no difference between medium- and low-dose UVA-1 phototherapy. These results are based on low-quality evidence.Limitations of data and analyses include risk of bias and imprecision (small number of participants or events and wide confidence intervals). We encourage multicentre RCTs to increase sample size and evaluate, with validated tools, different treatment responses according to the subtypes of morphea and age groups.

Challenges in the diagnosis and treatment of disabling pansclerotic morphea of childhood: case-based review.

Disabling pansclerotic morphea of childhood (DPMC) is a rare subtype of juvenile localized scleroderma (JLS) characterized by pansclerosis mainly affecting children under the age of 14. This aggressive disease has a poor prognosis due to the rapid progression of deep musculoskeletal atrophy resulting in cutaneous ulceration and severe joint contractures. We describe the challenges in treating a previously well 5-year-old male who has refractory symptoms of DPMC. Over the 29 months, since his initial presentation, we trialed over ten therapies. There was subjective improvement with prednisolone and mycophenolate mofetil (MMF). However, other therapies including biologics and tyrosine kinase inhibitors (TKI) were ineffective. The patient has been referred for hematopoietic stem cell transplant given ongoing disease progression. We conducted a literature search focusing on English articles with keywords including DPMC. Publications with limited information or describing cases aged 20 and above were excluded. Thirty-seven case reports were identified and the reported treatments were evaluated. Methotrexate and corticosteroids have been the most commonly utilized. MMF has been anecdotally effective. Biologics, TKI, and Janus kinase inhibitors lack evidence in DPMC, but have had demonstrated efficacy in similar pathologies including systemic sclerosis, and, thus, have been used for DPMC. Phototherapy has been documented to be reducing skin thickness and stiffness of plaques. Eventually, most children require multi-modal and high-dose immunosuppressive therapies to reduce the inflammation inflicted by the disease. Long-term antibiotics and nutritional support are important in the ongoing care of these patients.

Consensus-based recommendations for the management of juvenile localised scleroderma.

In 2012, a European initiative called Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile localised scleroderma (JLS) is a rare disease within the group of paediatric rheumatic diseases (PRD) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. This study aims to provide recommendations for assessment and treatment of JLS. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was formed, mainly from Europe, and consisted of 15 experienced paediatric rheumatologists and two young fellows. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using a nominal group technique. Recommendations were accepted if ≥80% agreement was reached. In total, 1 overarching principle, 10 recommendations on assessment and 6 recommendations on therapy were accepted with ≥80% agreement among experts. Topics covered include assessment of skin and extracutaneous involvement and suggested treatment pathways. The SHARE initiative aims to identify best practices for treatment of patients suffering from PRDs. Within this remit, recommendations for the assessment and treatment of JLS have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JLS throughout Europe.

Morphea: a practical review of its diagnosis, classification and treatment.

Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.

Bee venom acupuncture for circumscribed morphea in a patient with systemic sclerosis: A case report.

RATIONALE: Bee venom has been reported to demonstrate antinociceptive and anti-inflammatory effects in experimental studies, but there remain questions regarding the clinical use of bee venom, especially for scleroderma. This case report shows the successful outcome of bee venom acupuncture for circumscribed morphea in a patient with systemic sclerosis, which is considered to be a rare condition. PATIENT CONCERNS: A 64-year-old Korean woman had circular white areas (3 and 1 cm diameter) with severe itch in the right lateral iliac crest. Based on an initial diagnosis of systemic sclerosis (1 year prior to presentation at our clinic), she had been treated with painkillers, steroids, antitussive expectorants, and aspirin, with minimal effect on her recent skin symptoms. DIAGNOSES: In this study, the diagnosis of circumscribed morphea was based on localized skin symptoms of the patient with systemic sclerosis. INTERVENTIONS: The patient visited Gachon University Korean Medical Hospital for treatment of topical skin symptoms. After being evaluated for bee venom compatibility, she was administered subcutaneous bee venom acupuncture along the margins of the patches (superficial circumscribed lesions) using the shallow surround needling method twice per week for 1 week and then once per week for the following 3 weeks. OUTCOMES: Itch levels were evaluated before each treatment session: by her second visit, her itch had decreased from 8 to 3 on a 10-point numerical rating scale; by her sixth visit, her itch had decreased from 3 to 0. She did not experience adverse effects, and these improvements were maintained until the 2-month follow-up evaluation. LESSONS: Bee venom treatment demonstrates the potential to serve as an effective localized therapy for circumscribed morphea.

Comparing ultraviolet light A photo(chemo)therapy with Methotrexate protocol in childhood localized scleroderma: Evidence from systematic review and meta-analysis approach.

OBJECTIVE: Localized scleroderma is a skin fibrosing disorder that, if untreated, may result in severe disability. The purpose of this systematic review is to compare the present evidence concerning the effectiveness of Methotrexate versus phototherapy, alone or associated with Psoralen, in childhood localized scleroderma. METHOD: A systematic search between January 1996 and May 2017 was performed to identify studies investigating the efficacy of Methotrexate (MTX) or phototherapy (UVA) for treating localized scleroderma with onset ≤18 years. Due to a lack of validated clinical criteria, four clinical response criteria were used to assess the treatment efficacy as primary outcome. We determined a combined estimate of the proportion of children responding to MTX and UVA. RESULTS: A total of 19 studies was included (8 MTX; 11 UVA). In the methotrexate group, 193 children were included in the analysis; in the phototherapy group, a total of 48 treated children. For both groups age, disease subtype, glucocorticoids (GCs) use, and side effects of treatment were also analyzed. The meta-analysis suggested that UVA and MTX protocols have both a favorable effect in active lesions of childhood localized scleroderma. However, MTX resulted significantly superior to UVA, with or without Psoralen. CONCLUSION: Our study supports the combination of MTX and GCs in patients with a high risk of complication. Phototherapy with UVA1 could represent a therapeutic option in patients with limited scleroderma, where lesions do not cross joints and they do not lead to potential cosmetic changes.

Diagnostic criteria, severity classification and guidelines of localized scleroderma.

We established diagnostic criteria and severity classification of localized scleroderma because there is no established diagnostic criteria or widely accepted severity classification of the disease. Also, there has been no clinical guideline for localized scleroderma, so we established its clinical guideline ahead of all over the world. In particular, the clinical guideline was established by clinical questions based on evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guideline easy to use and reliable based on the newest evidence, and to present guidance as specific as possible for various clinical problems in treatment of localized scleroderma.

Morphea in Childhood: An Update. / Morfea en la infancia: actualización.

Morphea is an inflammatory, fibrosing skin disorder. When it occurs in childhood, it is also known as localized juvenile scleroderma. It is more common in girls and typically appears around the age of 5 to 7 years. According to a recent classification system, morphea is divided into 5 types: circumscribed (plaque), linear, generalized, pansclerotic, and mixed. Approximately 40% of patients present extracutaneous manifestations. Childhood morphea is treated with phototherapy, oral or topical calcitriol, topical tacrolimus 0.1%, methotrexate, topical or systemic corticosteroids, mycophenolate mofetil, bosentán, and topical imiquimod 5%. A variety of measuring tools are used to monitor response to treatment. Few prognostic studies have been conducted, but findings to date suggest that the disease tends to run a chronic or intermittent-recurrent course and frequently causes sequelae.