Form Of Localized Scleroderma Research Articles

Localized scleroderma

Localized scleroderma (LS), also called circumscribed scleroderma or morphea, comprises aheterogeneous group of diseases that can be classified into four subtypes: limited, linear, generalized, and mixed LS. All manifestations are primarily due to chronic progressive fibrosis of the skin or structures close to the skin. Involvement of internal organs or the transition to systemic sclerosis is excluded by definition. Adistinction is made between forms that primarily affect the skin (up to the dermis) or that severely involve subcutaneous fat tissue, muscle fascia or muscles. Adetailed examination is required for clinical diagnosis. In order to improve comparability of findings, photo documentation and the use of clinical scores should be carried out. For superficial subtypes the use of topical glucocorticosteroids, calcineurin inhibitors or phototherapy is initially recommended, whereas for severe forms with deep involvement or overall therapy refractoriness, the diagnosis should first be expanded and systemic therapy initiated at an early stage. Especially, in cross joint or extremity-dominant forms of linear LS or in cases with head and neck involvement, such as en coup de sabre, Parry-Romberg syndrome and other subtypes with aprominent musculoskeletal affection, an MRI examination should be arranged. Depending on location, an ophthalmological, neurological, orthodontic, rheumatological or orthopedic consultation may be necessary. For systemic therapy, methotrexate alone or in combination with systemic glucocorticosteroids as pulse therapy is recommended as first-line treatment.

MODERN TREATMENT AND CORRECTION OF THE CONSEQUENCES OF LOCALIZED SCLERODERMA

Localized scleroderma (LS) is an inflammatory sclerosing disease of the skin and subcutaneous tissues associated with their atrophy. Depending on the subtype, severity of the disease and localization of the lesion, involvement in the pathological process of adipose tissue, muscles, joints and bones, but not internal organs, is noted. The annual primary incidence of drugs in childhood is 3.4 cases per 1,000,000 child population; in females, the disease occurs 2.6–6 times more often. The wide range of clinical manifestations of the disease has led to the emergence of a large number of different classifications, which take into account the severity, prevalence and depth of the fibrosis process, as a result of which five main clinical forms of LS are distinguished: limited, generalized, linear, deep and mixed. The forms of drugs are not mutually exclusive, since the same patient may experience different manifestations of the disease. One of the most common forms of LS in childhood is the linear form, which is observed in approximately 40–70% of children.

Face Lesions in En Coup De Sabre Scleroderma in Children: Modern Treatment and Outcomes Improvement

Localized scleroderma (LS) is an inflammatory sclerosing disease of the skin and subcutaneous tissues associated with its atrophy. Commonly, LS is a benign self-limited disease, although, the chronic form of this disease is recurrent. Particular attention is paid to the research of treatments methods that could eliminate not only immune-mediated mechanisms, but also its outcomes (such as gross cosmetic defects on the face), which negatively affect child’s physical and psycho-emotional development. Recently, fat transplantation efficacy has been studied as it can restore the volume and improve skin quality. This article presents the results of such surgery in a patient (15 years old) with linear form of LS.

Sun Sign in the Ultrasound Diagnosis and Activity Assessment of Morphea

Morphea, a localized form of scleroderma, is a chronic inflammatory autoimmune disease of the skin. Color Doppler Ultrasound has been reported as a reliable tool to assess the activity of the disease. With histologically confirmed cases, this case series describes a new ultrasound sign consisting of a hyperechoic halo surrounding superficial subcutaneous veins of the extremities in transverse view, named the sun sign. This sign can help diagnose morphea in the inflammatory phase and correlate in pathology with perivascular infiltrates surrounding superficial subcutaneous veins.

Proliferative function of endothelium in local scleroderma, gender and age characteristics of the disease

Objective — to investigate the variability of vascular endothelial growth factor (VEGF) in localized scleroderma (LS), characteristics of the disease in different age groups. Materials and methods. The study included the clinical evaluation of 78 patients (27 men, 51 women) with localized scleroderma aged (43.2 ± 7.28) years. All patients underwent clinical, laboratory, and enzyme immunoassay investigation of VEGF. The criteria for inclusion in the study were: the presence of localized scleroderma and the absence of signs of a systemic process. Results and discussion. It has been proven that localized scleroderma is gender­related, more often recorded in women (65.4 %, p < 0.05), with an attributive risk of 50.0 % compared to men. Late diagnosis of the disease was proven in 78.2 % of patients. The age and gender characteristics of this pathology were determined — men are more likely to get sick at a young age (up to 20 years; 22.2 % of the surveyed), however, women over the age of 55 prevail (p < 0.05). Women sought dermatological help earlier compared to men (29.4 %; p < 0.05), but had a higher risk of early progression (p < 0.05). Among the clinical forms of localized scleroderma, most of the cases (70.5 %) were of the plaque form. In 15.4% of cases, a linear form was diagnosed, in 9.0% ­ lichen sclerosus, in 5.1 % — Pasini—Pierini idiopathic atrophoderma. The plaque form of LS was significantly more often detected in women (80.3 %), and the linear form and lichen sclerosus — in men (22.2 % each). The average number of lesions was 2.8 ± 1.12. Three lesions were found in almost half (43.6 %) of the patients, one lesion – in 23.1%, two lesions – in 16.7%, four lesions – in 11.5%. Five or more lesions were present in 4 (5.1 %) patients. It has been found that the highest content of VEGF was observed in classic plaque form (p < 0.05), the lowest — in Pasini—Pierini idiopathic atrophoderma (p < 0.05). U­shaped age dependence of the disease was determined: high content of VEGF­A in patients under the age of 20 (p < 0.05), with a significant decrease of VEGF­A in patients aged 20—35 years (p < 0.05) and further increase in those aged 35 to 55 years (p < 0.05). It was established that VEGF­A level increased at patients with early progression of the disease — in the first 2 years after the start of clinical manifestations against the group of patients with slow progression — more than 6 years (p < 0.05). In assessment of gender dependence, we found a higher content of VEGF­A in female patients compared to male patients (p < 0.05). Conclusions. Localized scleroderma has gender and age­related characteristics and is 50.0 % more often recorded in women. Among men, young patients predominate. Studying the pathogenesis of LS and assessing the proliferative function of the endothelium of the disease will make it possible to predict the course of the disease, improve the diagnosis and treatment.

Treatment of En Coup de Sabre Deformity with Fat Grafting and Demineralized Bone Matrix: A Case Series

En coup de sabre deformity (ECDS) is a form of localized scleroderma in the frontoparietal region caused by progressive subcutaneous tissue atrophy and bony defect. Although ECDS involves two layers, skin/subcutaneous tissue and bone, the existing literature mainly focuses only on treating the skin/subcutaneous tissue layer. In this case series, we aimed to propose a novel approach that includes the combined use of fat grafting and demineralized bone matrix (DBM). Four patients with ECDS deformity, operated between February 2016 and October 2018, were retrospectively evaluated. All the patients were treated with the novel approach. Patients were evaluated with localized scleroderma scale and computed tomography (CT) scan in the preoperative period and at the annual follow-up. We observed remarkable improvement in the localized scleroderma scale including appearance, palpation, and size scores in all patients at the annual follow-up. CT scans at the annual follow-up revealed new callus formation at the bony defect area in all patients. Reinforcing fat grafting with DBM could promote healing of the bony and skin/subcutaneous tissue defects associated with ECDS.

Congenital Morphea: Two Case Reports

Congenital morphea is a rare and underestimated form of localized scleroderma that presents at birth. From the five known subtypes, linear and plaque morphea are the most common and benign forms and are both described in these cases. Extracutaneous manifestations are rare.

Natural history and extracutaneous involvement of congenital morphea: Multicenter retrospective cohort study and literature review.

Congenital morphea is a form of localized scleroderma that presents at birth. There is limited information on its presentation and progression. Patients with congenital morphea were identified from five pediatric dermatology and rheumatology tertiary care centers in Canada, the United States, and Italy from 2001 to 2016. Cases from the literature were identified by searching Ovid (EMBASE and MEDLINE) from inception to June 30, 2017. Disease characteristics and prevalence of extracutaneous involvement were analyzed. Thirteen patients were identified from the five centers, and 13 cases were described in the literature, representing 25 patients, with one duplication. Fourteen patients (56%) were female. Median age at diagnosis was 2.9years (interquartile range 1.2-5.1years). Linear morphea, including en coup de sabre and Parry-Romberg syndrome, was the most common subtype observed (n=19, 76%), followed by circumscribed (n=5, 20%), generalized (n=2, 8%), and mixed (n=2, 8%). The face (n=14, 56%), scalp (n=8, 32%), and trunk (n=6, 24%) were the most common locations affected. Most lesions were active at diagnosis (n=19, 76%), but all patients with follow-up later became inactive. Extracutaneous involvement was seen in 12 (48%) patients, all of whom had linear morphea. Musculoskeletal sequelae were seen in those with linear morphea of the extremities (4/5, 80%), and neurologic involvement was seen in those with linear morphea of the head (8/13, 62%). Congenital morphea is associated with extracutaneous manifestations and delayed diagnosis. More research is needed to determine whether early recognition, monitoring, and treatment can alter the disease course.

S06. A case of linear scleroderma en coup de sabre and epilepsy: Manage the sabre to control the seizures

Introduction Linear scleroderma en coup de sabre (ECS) is a rare form of localized scleroderma (0.4–2.7/100,000 people) ( Peterson et al., 1997 ) that has characteristic cutaneous involvement of the forehead and scalp ( Holland et al., 2006 ) and is named in reference to its “stroke of a sabre”-like appearance. It typically has onset in the pediatric population and has a higher association with extracutaneous manifestations than other forms of localized scleroderma. Focal epilepsy is the most commonly reported extracutaneous manifestation of ECS, seen in 42% of reported cases ( Doolittle et al., 2015 ). In one study, 7 of 7 patients with ECS associated epilepsy had abnormal brain imaging ( Amaral et al., 2013 ). Methods Ms. J is a 24 year old right handed woman with history of ECS diagnosed in 2007 involving the left forehead, refractory left temporal lobe epilepsy with onset in 2008, and a left anterior temporal lobe lesion that was resected in 2008 with reported pathology of arteriovenous malformation (AVM). Ms. J has undergone extensive evaluation by physicians in the departments of neurology and rheumatology at the Mayo Clinic since 2013, including two epilepsy monitoring unit (EMU) evaluations, ictal-SPECT scan, serial brain MRI and head and neck MRA studies, serological testing, and CSF testing. She has been treated with various antiepileptic medications, and methotrexate and mycophenolate mofetil have been used to manage her ECS. Results Preoperative imaging of the left anterior temporal lobe lesion was retrospectively reviewed by the Mayo Clinic neuroradiology department and is not suggestive of AVM; request for pathology slides from the outside facility has been unsuccessful. Serial imaging since the resection shows new T2 hyperintensity, microhemorrages on susceptibility sequences, and scattered enhancement in the mid and posterior left temporal region, identical in appearance to the resected anterior temporal lobe lesion. Ms. J enjoyed 2 extended periods of seizure freedom, 2008 – May, 2011, and November, 2013 – November, 2016. Her seizure control correlated with treatment of her ECS: she reliably took methotrexate from 2008 – February, 2011 and mycophenolate mofetil from September, 2013 – August, 2016. She restarted mycophenolate mofetil in Jaunary, 2017 and has been seizure free since July (except for an EMU admission October, 2017 on reduced antiepileptic medications). She has been treated with a combination of lamotrigine, levetiracetam, and oxcarbazepine, currently all three, and was maintained on antiepileptic medications since 2008. Conclusion This case illustrates the importance to recognize ECS as a cause of focal epilepsy and brain imaging lesions, and suggests a need for ECS management for seizure control. Proper diagnosis is needed to avoid ineffective brain surgery and to prompt appropriate ECS treatment.

TUMOR-ASSOCIATED LOCALIZED SCLERODERMA IN CONJUNCTION WITH ACQIRED DISSIMINATED FORM OF VITILIGO: A CLINICAL CASE

We are presenting the clinical and laboratory case of management of 62 years old patient with combined dermatological tumor-associated diseases: localized form of scleroderma, acquired disseminated form of hypopigmentation (vitiligo). The patient initial visit in a medical dermatological facility was due to the emergence of a consistent following complaints such as the sudden appearance of alterated skin sites in the left breast and some later the occurrence of rushes on the skin on the chest area and on the anterior surface of the abdomen. Upon further examination of the patient have been verified malignant tumor of the left breast, therefore appropriate (complex) treatment have been conducted in a medical institution of oncology service. After 1.5 years after the sectoral mastectomy the patient appealed to the dermatologist again with a complaints to the renewed activity of scleroderma process on the skin of the left breast. On the background of activation of dermatological symptoms the patient have been diagnosed recurrent tumor in the left breast. This clinical case is a multipurpose: first of all, it is demonstrates the importance of the principle of oncological vigilance in multidisciplinary daily medical practice which would improve early diagnosis and, consequently, treatment of the underlying disease; secondly, the present clinical case suggests an unexpected paraneoplastic contour of the diseases which are unpopular in this category previously, but generally frequent in the population.

Morphea and vitiligo-A very uncommon association

Morphea represents a localized form of scleroderma where there is predominant skin involvement, with occasional involvement of subjacent muscles. However, it usually spares the internal organs [1]. Morphea has been associated with several autoimmune diseases like mixed connective tissue disease, dermatomyositis, pemphigus, myasthenia gravis, bullous pemphigoid, systemic sclerosis, Hashimoto’s thyroiditis, etc [2,3]. The association of morphea with vitiligo has been rarely reported. In this article we report a 29 year female with concomitant vitiligo and plaque type morphea over her lower back.

AB0651 Eosinophilic Fasciitis (Schulman's Disease): Clinical Spectrum, Response to Treatment and Long Term Outcome in A Series of 18 Spanish Patients

BackgroundEosinophilic fasciitis (EF) is a rare disorder characterized by symmetrical skin induration of the limbs, peripheral eosinophilia, and characteristic histopathological changes in the muscular fascia. Although it has been considered...

A Child With Morphea in a Zosteriform Distribution

Morphea is a localized form of scleroderma which is characterized by sclerotic plaques, limited to the skin. Although its cause is unknown, various (genetic, infectious and autoimmune) mechanisms have been suggested. It is more common among children and young women. Although clinical outcome is good, sometimes it can be prominent. Morphea have five subtypes, which are known as plaque, generalized, bullous, deep and linear forms. Zosteriform morphea is a recently described pattern and it is rarely observed. A six years old female, was admitted with multiple brown and white asymptomatic plaques arranged in a zosteriform pattern, confined to the right back since two months, without history of herpes zoster at the same location. We aimed to report the first pediatric case of morphea with zosteriform pattern in the absence of herpes zoster history.

Systemic sclerosis with keloidal nodules

Nodular sclerosis is a rare form of presentation related to both systemic and localized forms of scleroderma. We describe the case of a patient with nodular sclerosis in order to alert the medical community to recognize this entity.

Localised Scleroderma-Patchy Type Morphea

Background: Scleroderma comes in two main forms: systemic and localized. Morphea is a localized form of scleroderma and affects primarily the skin. The cause of morphea is unknown. Literature focuses on Borrelia burgdorferi as a possible etiologic agent for morphea. Methods: It is a case presentation of 8 years old female. The first cutaneous changes have occurred three months prior to hospitalization with appearance of a solitary red patch surrounded by a violet ring in suprapubical region. Within a short time several other patches similar to the first one have been appeared close to the first one and one on the spine. A year ahead she experienced the tick bite on the head. Conclusions: Although the etiology of morphea is unknown the Borrelia origin of morphea was verified by the presence of antibodies against Borrelia Burgdorferi. The data obtained in this study suggest that Borrelia Burgdorferi may play a role in the etiopathogenesis of disease.

Morphea and other localized forms of scleroderma

Morphea, also known as localized scleroderma, is a disorder of excessive collagen deposition leading to thickening of the dermis and/or subcutaneous tissues and may cause significant morbidity. This review will describe new developments in the evaluation and management of morphea as well as its pathophysiology. The reader will be able to apply these findings to patient management. The recent development of validated outcome measures (i.e. the localized scleroderma cutaneous assessment tool) as well as consensus treatment recommendations provide a platform for collaboration among specialties to develop both standardized assessment tools and therapeutic trials. New studies have also begun to investigate the immunological underpinnings of morphea. The promise of evidence-based treatments for morphea in the near future will provide better care for patients with morphea and understanding its pathophysiology will lay groundwork for the development of new treatments.

Brain cavernomas associated with en coup de sabre linear scleroderma: Two case reports

Linear scleroderma is a form of localized scleroderma that primarily affects the pediatric population. When it occurs on the scalp or forehead, it is termed "en coup de sabre". In the en coup de sabre subtype, many extracutaneous associations, mostly neurological, have been described. A patient with linear scleroderma en coup de sabre was noted to have ipsilateral brain cavernomas by magnetic resonance imaging. Using a worldwide pediatric rheumatology electronic list-serve, another patient with the same 2 conditions was identified. These two patients are reported in this study. Consideration of neuroimaging studies to disclose abnormal findings in patients with linear scleroderma en coup de sabre is important for potentially preventing and treating neurological manifestations associated with this condition.

Morphea as a Consequence of Accelerated Partial Breast Irradiation

Morphea is a localized form of scleroderma usually unaccompanied by the typical systemic stigmata that characterize progressive systemic scleroderma. It rarely manifests at the site of whole breast external-beam radiation therapy. We present an unusual case of radiation-induced morphea (RIM) that occurred after accelerated partial breast irradiation (APBI) using intracavitary Contura brachytherapy. A 65-year-old white woman was treated for stage IIA invasive ductal carcinoma of the left breast with APBI to a dose of 34 Gy in 3.4-Gy fractions twice daily over the course 5 days with intracavitary brachytherapy. At 1.5 years after completion of APBI, the patient developed an area of tenderness, erythema, and induration at the site of irradiation. A skin biopsy was consistent with morphea. To our knowledge, this is the first case of RIM confined to the area of APBI.

Sklerodermie

Scleroderma (synonyms: systemic sclerosis, systemic scleroderma) is a systemic disease which affects the skin as well as internal organs such as the lungs, gastrointestinal tract, kidneys, and the heart. Pathogenetically a distinction should be made between uncontrolled formation of extracellular matrix proteins (fibrosis) and vasculopathy. In addition to organ fibrosis, this leads to a clinical picture of vascular manifestations. These include fingertip ulcers, pulmonary arterial hypertension, and acute renal crisis. Localized forms of scleroderma, such as morphea, which do not involve organ complications, should be differentiated from systemic sclerosis. Due to its clinical heterogeneity and high rate of morbidity and mortality, systemic sclerosis poses an enormous diagnostic and therapeutic challenge in everyday clinical practice. This review article summarizes the current status of classification and epidemiology, pathogenesis, and the most important clinical manifestations such as interstitial fibrosis, pulmonary arterial hypertension, acute renal crisis, and peripheral vasculopathy and provides an overview of current and future treatment options.

Systemic Sclerosis and Cancer

To review recent advances and current controversies on the association between systemic sclerosis (SSc) and cancer, PUBMED was searched from 1966 to the present using the terms: systemic sclerosis, cancer, morphoea, sclerotic diseases. Malignancies, mainly in lung and breast, coexist with idiopathic SSc or with SSc-like disorders, but not with localized forms of scleroderma (morphoea), with the exception of squamous cell carcinoma in patients with pansclerotic morphoea and skin ulcers. The mechanisms connecting SSc and malignancies are unknown. The occurrence of different cancer types with SSc or SSc-like disorders suggest different underlying mechanisms, including altered immune response, common genetic and environmental links, disease-dependent factors, tumor-derived biologic substances and therapies. The process of sclerosis itself may favour cancer in certain sites, and a reaction between T cells and neoantigens formed during irradiation has been suggested to explain the frequent development of morphoea after breast irradiation. Radiotherapy, especially when used for breast cancer, may trigger idiopathic SSc or morphoea and influence the severity of preexisting SSc, with the consequence that SSc is considered a relative contraindication to breast radiotherapy. In conclusion, cancer and SSc may be associated, but it is still controversial as to whether there is a causal relationship. Continuing interest in these associations, in particular in the different modalities of associations, may help to understand the underlying biological mechanisms and to identify patients at risk.