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Background There are still few dermatological studies on morphea. We evaluated the epidemiological and clinical features and management of pediatric morphea, reporting dermatologists experience. Methods A multicentre retrospective observational study was carried out on the epidemiological and clinical features and management of the disease between 01/01/2009 and 01/10/2014 in 10 Italian Dermatological Units. Results We collected the data of 69 children affected by: circumscribed morphea (39.1%); linear morphea of trunk and limbs (14.5%); en coupe de sabre morphea (ECDS) (14.5%); progressive facial hemiatrophy (8.7%); generalized form (18.8%); mixed morphea (4.4%). The mean age at onset was 6.86±3.21 years, mainly between 2 and 8 years, but is statistically significantly lower for ECDS (4.5±3.03). Localizations were: head/neck (30.4%), limbs (26.1%), trunk (14.5%), 2 or more sites (29%), most often the trunk plus limbs. Extracutaneous manifestations were observed in 26.1% patients. 10 patients presented a second autoimmune disorder. Treatments were topical in 26.1% cases and systemic (alone or associated with topical treatments) in 68.1%. Conclusions There was a lack of uniformity in the management of patients and an increasing awareness of dermatologists on the use of systemic therapies, in particular of methotrexate, which is no longer exclusive to rheumatologists. Methotrexate causes stabilization and improvement of the clinical signs, but topical creams are still considered adjuvant or maintenance therapies during/after the use of systemic drugs.

Objective: To study the clinical characteristics and prognosis of morphea in Thai adult patients. Methods: The medical records of 81 morphea adult patients who visited Siriraj Hospital, Thailand, between 2006 and 2015, were retrospectively reviewed. The demographics, clinical features, treatments and outcomes were analyzed. Clinical improvement was categorized as excellent (>80%), partial (1%–80%), and no response. Results: Circumscribed morphea was the most common subtype (34, 42%) of the 81 patients, followed by linear morphea (21, 26%), generalized morphea (14, 17%), en coup de sabre (11, 14%) and Parry–Romberg syndrome (1, 1%). Systemic treatment and ultraviolet A (UVA)1 phototherapy had satisfactory outcomes in 83% and 79% of cases, respectively, providing a partial to excellent response. For overall remission, one year after the treatment, 30% of patients achieved a partial to excellent response. After 2 years, this proportion rose to 50%. The median time to clinical response was 24 months. Conclusion: Morphea is a difficult-to-treat dermatosis, with the majority of the patients having a partial clinical response and a high recurrent rate. Combination of treatment might be a worthy option.

Background The exact pathogenesis of morphea is poorly understood, and only a few clinical or histopathological studies have been conducted in Asian patients. Objective The aim of this study was to identify the clinical and histopathological characteristics of morphea in Korean patients. Methods We retrospectively reviewed the medical records of 101 patients whose clinicopathologic findings were compatible with morphea and analyzed demographic characteristics, number of lesions, disease subtype and histopathological findings. Results Overall, circumscribed morphea (52.5%) was the most common clinical type, followed by linear (28.7%), mixed (13.9%) and generalized (5.0%) type. Disease duration was positively correlated with increased thickness of the skin in 54 patients (p

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.

Juvenile localised scleroderma is a rare childhood disorder of the immune system and connective tissue with unknown etiology. There are different types of localised scleroderma, including linear scleroderma (where the lesion appears as a line or streak) and plaque or circumscribed morphea (where the lesion appears as a roundish lesion). The present report describes the case of a 10-year-old girl, who presented with a history of linear scleroderma with nose, oral and dental involvement, and outlines the diagnosis of the case based on the clinical presentation, pathology, X-ray and cone beam computed tomography images. The treatment strategy that was selected for the patient is additionally discussed.

Introduction: Morphea, also referred to as localized scleroderma, is a rare fibrosing skin disorder of undetermined cause. Aim: We report our single-center experience with morphea. Material and methods: The study included 53 patients who were diagnosed with morphea by histopathology in our department between 2010 and 2015. Study data were collected retrospectively from the records of morphea patients. Results: The study included 53 patients (38 women, 15 men), and median age at onset was 39.0 (range: 8–85) years. Thirty (56.6%) patients had circumscribed morphea, 15 (28.3%) had generalized morphea, and 7 (13.2%) had linear morphea. One patient had mixed variant morphea (generalized, pansclerotic and linear morphea). ANA positivity was detected in 12 (22.6%) patients, but analysis for an association between the presence of ANA and morphea types, patients’ characteristics did not reveal any significant associations. We did not observe any extracutaneous manifestations in patients during follow-up period. There were 2 of 53 patients who had concomitant autoimmune disorder including vitiligo and spondyloarthritis. Thirty (56.6%) patients received only topical treatment. The patients with clinical improvement who were treated with systemic therapy received methotrexate (26.4%), colchicine (9.4%), mycophenolate mofetil (5.7%) and prednisolone (1.9%). Conclusions: Our results related to the demographic data of the patients and morphea types were consistent with the literature. On the other hand we observed that methotrexate was mostly used as an effective treatment option for generalized morphea.

Although a few reports have noted the concurrent presentation of morphea and vitiligo at distinctly separate sites in the same patient, it is extremely rare that these two conditions occur at the same sites in a patient. We report the case of a 10-year-old Korean girl with morphea and vitiligo and those lesions occurred at the same sites and progressed simultaneously. An autoimmunity and a cutaneous mosaicism was considered to be involved in such an unique presentation as the pathogenesis is concerned. (Ann Dermatol 30(6) 708∼711, 2018)

Dear Editor: A 10-year-old girl presented with a round, brown to whitish, atrophic plaque on the right side of her back, which she has had for 5 years. Histologic findings showed thick collagen bundles with lymphocytic infiltrations around the vessel in the dermo-subcutaneous tissue, which are consistent with morphea. Only topical calcipotriol was used due to the limited localization of the plaque and the young age of the patient. However, after 6 months, a new morphea lesion developed on the left side of the back and linear scleroderma occurred on the forehead. The patient was diagnosed with generalized mixed-type morphea, with two circumscribed morphea on the right and left sides of the back (Fig. 1A) and linear scleroderma on the forehead (Fig. 1B). Hypopigmented lesions were also present on her left leg and on the left side of the trunk (Fig. 1C). Based on Wood's light examination and clinical findings, the patient was diagnosed with segmental vitiligo (SV). Fig. 1 (A) Two circumscribed morphea on the right and left sides of the back. (B) Linear scleroderma on the left side of the forehead. (C) Vitiliginous lesions on the left side of the trunk. The vitiliginous lesions and linear scleroderma on the forehead showed progression despite the combination therapy using intermittent oral steroid, topical calcineurin inhibitor, and narrowband ultraviolet B phototherapy for 1 year. To block the progression of morphea, hydroxychloroquine sulfate (200 mg/day) was used. Skin lesions of morphea and vitiligo stabilized within 6 weeks. In addition, the vitiliginous lesions showed 70% repigmentation after 5 months from start of medication. Skin lesions of morphea also showed no more progression. The patient tolerated the 7-month treatment period with no side effects (Fig. 2). Fig. 2 Flow diagram during the follow-up period. NBUVB: narrowband ultraviolet B. Although the pathogenesis of vitiligo remains unknown, an immunological mechanism is strongly considered. However, SV is associated with a neurogenic mechanism. Furthermore, an immune-mediated mechanism known as the three-step theory has been reported to be involved in the pathogenesis of SV, according to a case report showing simultaneous occurrence of SV, alopecia areata, psoriasis, and halo nevus1. The association of SV with scleroderma has been rarely reported2. Bonifati et al.3 reported the case showing linear scleroderma on the left limb and homolateral SV on the trunk. Contrary to previous reports, our patient had generalized morphea lesions on the right and left sides of the back and linear scleroderma on the left side of the forehead, which was on the same side of the body where SV was located. Our case study suggested that hydroxychloroquine is effective not only in blocking the progression of scleroderma4 but also in treating vitiligo. Walsh et al.5 reported two systemic lupus erythematosus patients with exextensive widespread vitiligo-like depigmentations who showed rapid responses to hydroxychloroquine. Although we cannot exclude the possibility of coincidental development of SV and scleroderma, our case suggested that vitiligo and scleroderma that have the same autoimmune background can occur at the same time because they occurred during the same period and progressed in parallel for several years. In addition, this concomitant presence could support the theory that SV is associated with an autoimmune background. Moreover, our report suggested that an antimalarial drug could be used as a promising option for the treatment of vitiligo.

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.

Morphea is a rare fibrosing disorder of the skin and underlying tissues. Circumscribed morphea presents with less than three discrete indurated plaques and breasts are commonly affected in women. We report the case of a 12-year-old female with a right infra-areolar, nontender, brownish patch and asymmetry of the right breast with 2 years of evolution. Skin biopsy showing thickening of the dermal collagen bundles confirmed the clinical diagnosis of morphea. After a 3-year follow-up period without progression of disease, reconstructive surgery is scheduled. Plaque morphea can involve all layers of the skin but associated breast deformity is rare. It can mimic benign and malignant breast disorders justifying the benefit for early tissue biopsy. Breast morphea generally has a good prognosis but hyperpigmentation and breast deformity in young girls have been rarely described. An early diagnosis can possibly lead to a therapeutic intervention with a different outcome, as it can be the source of severe psychological and social issues in a delicate period of development such as adolescence.

Localized scleroderma (LS), also known as morphea, is the most frequent form of scleroderma in childhood and is grouped into five subtypes: circumscribed morphea, linear scleroderma, generalized morphea, pansclerotic morphea, and a mixed subtype, where a combination of two or more of the previous subtypes is present. Linear scleroderma is the most frequent subtype in childhood, and when it involves the face (LSF), it may cause aesthetic and functional abnormalities, sometimes complicated by hemifacial atrophy.

We report 2 patients with typical clinical findings of circumscribed morphea who on histopathologic examinations had histiocytes ("floating sign") surrounding individual collagen fibers in the dermis in addition to the key histologic findings of morphea. To our knowledge, there are no previous reports in the medical literature of such a phenomenon. Histopathological findings in idiopathic morphea and morphea-like conditions are reviewed.

Background/Purpose: Localized scleroderma (LS) is a chronic inflammatory and fibrosing skin disease. We present baseline data on the juvenile LS (jLS) cohort from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) registry, a multicenter observational pediatric rheumatic disease registry. Methods: Descriptive statistics were used for demographic, clinical and laboratory features. Data analysis included the two-sample t-test, chi-square test, Fisher's exact test, and analysis of variance as appropriate. Results: Of 259 children in the database, 78% were female and 81% were Caucasian. Mean age at onset was 8.3 yr (± 4.2). Mean age at first pediatric rheumatology (PRH) evaluation was 9.5 yr (± 4.2), yet 37% had ≥5 yr delay from onset to first PRH visit. Linear scleroderma (LiS) was the most common subtype (54%), followed by circumscribed morphea (CM) (15%), generalized morphea (GM) (8%), eosinophilic fasciitis (2%), and pansclerotic morphea (1%). 20% of children had mixed subtype, and LiS-CM was the most frequent combination (60%). Among LiS patients with face-scalp localization (40%), neurologic and ocular diseases were reported in 7% and 4%, respectively. ANA positivity was found in 50% tested and was not associated with subtype, age at onset, extracutaneous manifestations, or features of disease damage. Children with new lesions were more likely to have an elevated creatine kinase (CK) (p=0.02) or aldolase (p=0.02); muscle atrophy (p=0.04) and extremity shortening (p=0.02) were also associated with an elevated CK. Children with any functional limitation (baseline worst ever ACR functional class II, III, and IV) (28%) had earlier first PRH visit (mean 0.88 yr ± 0.89) compared to those without limitation (class I) (mean 1.4 yr ± 1.8, p=0.03). The association was also significant when evaluating ≥1 yr (p=0.04), ≥2 yr (p=0.02), and ≥5 yr delay (p=0.02) in first PRH visit. Poorer function also correlated with presence of muscle atrophy, joint contracture, and extremity shortening (all p

Introduction Scleroderma is an autoimmune disease that involves the connective tissue characterized by skin fibrosis, classified as localized and systemic (participation of one or more internal organs). The primary objective of this study is to describe and analyze the clinical and laboratory findings in a group of children diagnosed with scleroderma at a referral hospital. Material and methods Extraction of data from clinical charts of children with scleroderma in the rheumatology department at the Hospital Infantil de Mexico Federico Gomez, between January 2000 and December 2007. Results Sixty-two patients were included in the group. All of them completed the classification criteria for juvenile sclerodema, both systemic and localized. The mean age at diagnosis was 7.8 (1–14) years. The mean time from disease onset to diagnosis, based on clinical manifestations, was 23 months. The lesions found were: linear scleroderma (42%), mixed morphea (22%), circumscribed morphea (19%), generalized morphea (13%), and panclerotic morphea (4%). Involvement associated with systemic scleroderma was gastrointestinal 100% (18 patients), pulmonary 100% (18/18), Raynaud's phenomenon 89% (16/18), proximal sclerosis 89% (16/18), sclerodactilia 67% (12/18), joint pain 28% (5/18), calcinosis 56% (10/18). Positive antinuclear antibodies (ANA) were present in 14/62 (23%) patients (10 with systemic range and 4 localized), antiSCL 70 in 2/62 (4%) cases. The most common drug used was methotrexate. Conclusion The most common skin lesions found were linear morphea, followed by the mixed and circumscribed types. In systemic scleroderma the most involved systems are the gastrointestinal, respiratory, and vascular (associated with Raynaud's phenomenon). There is a special need for knowledge of this disease in first contact physicians for a faster and better diagnosis and treatment, in order to avoid complications. It is also necessary to improve resources in developing countries for complimentary studies, classification, treatment, and follow-up.

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/m 2 , 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/m 2 . 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

Juvenile localized scleroderma, also known as morphea, is the more frequent subtype of scleroderma in childhood. It comprises a group of distinct conditions which involve the skin and subcutaneous tissues. They range from very small plaques of fibrosis involving only the skin, to diseases which may cause significant functional and cosmetic deformity. The most widely used classification divides JLS into five general types: circumscribed morphea (CM), linear scleroderma, generalized morphea (GM), pansclerotic morphea and the new mixed subtype where a combination of two or more of the previous subtypes is present. Circumscribed morphea (CM) is characterized by oval or round circumscribed areas of induration surrounded by a violaceous halo. When there are four or more plaques with individual plaques that are larger than 3 cm and they become confluent involving at least two out of seven anatomic sites (head-neck, right upper extremity, left upper extremity, right lower extremity, left lower extremity, anterior trunk, posterior trunk) it is called Generalized Morphea (GM). Linear scleroderma, the most common subtype in children and adolescents, is characterized by one or more linear streaks that can extend through the dermis, subcutaneous tissue, and muscle to the underlying bone, causing significant deformities. The upper or lower extremities can be affected but also the face or scalp, as in the en coup de sabre variety (ECDS). The Parry Romberg syndrome (PRS), characterized by hemifacial atrophy of the skin and tissue below the forehead, with mild or absent involvement of the superficial skin is considered the severe end of the spectrum of ECDS and for this reason is included in subtype of linear scleroderma Pansclerotic morphea, an extremely rare but severe subtype, is characterized by generalized full-thickness involvement of the skin of the trunk, extremities, face and scalp with sparing of the fingertips and toes. A recent multinational study reported that almost one fourth of the patients present extra-cutaneous manifestations such as arthritis, neurological findings, associated autoimmune conditions or ocular abnormalities. Antinuclear antibodies (ANA) are present in more than 40% of patients with JLS. The management of JLS is challenging and the detection of disease activity and progression remains a fundamental problem. Clinical examination is subjective, classical skin scoring methods, utilized in the assessment of systemic sclerosis, cannot be applied. Among the new tools which have been proposed for the assessment of the skin lesions, infrared thermography (IRT), computerized skin score (CSS), ultrasound (US) and magnetic resonance imaging (MRI) are those most frequently used. Over the years, many treatments have tried for localized scleroderma. Circumscribed morphea generally is of cosmetic concern only, and therefore treatments with potentially significant toxicity are not justified. When there is a significant risk for disability, such as in linear and deep subtypes, systemic treatment methotrexate (MTX) in combination with corticosteroids should be considered.