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There are several types of morphea with different levels of connective tissue involvement and morphological manifestations. In this mini review, it was pointed out the most important morphological and clinical aspects of localised scleroderma in the oral cavity. The case presented in this article supports the scientific information and is described with details. The morphea of mucous membrane which was clinically suspected, was proved by histopathological examination of the sample. The unusual location of the local findings posed a diagnostic challenge. The case history should be significant due to the low number of studies. The special attention should be taken to match the clinical with pathomorphological picture in localised scleroderma diagnosis and treatment when the involvement of skin and oral mucosa is.

BACKGROUND Linear scleroderma is a rare connective tissue disease characterized by thickening of the skin due to excess collagen deposit. It is the most frequent type of localized scleroderma in the pediatric population (40%-70%), and it usually affects the extremities and head. Linear scleroderma can cause growth disturbances, extremity length discrepancy, and debilitating joint contractures. The aim of this study was to describe the surgical outcomes of microsurgical flap reconstruction of linear scleroderma in the pediatric population. METHODS A retrospective review of all children with linear scleroderma was performed at our institution. Patients less than 18 years of age with clinical diagnosis of linear scleroderma and who underwent microsurgical flap reconstruction for correction of their scleroderma were included. Fat grafting procedures were excluded. Patient demographics and surgical outcomes were collected. RESULTS A total 7 patients underwent microsurgical flap reconstruction for treatment of their linear scleroderma producing debilitating soft tissue contractures and preventing ambulation or elbow extension. Six patients had scleroderma involving the lower extremity and 1 involving the upper extremity. Three anterolateral thigh and 4 latissimus dorsi flaps were performed. The median age at surgery was 7 years (Q1-Q3, 5-11), and the median body mass index was 21.3 kg/m2 (Q1-Q3, 16.5-22.1). The median follow-up was 121.1 months (Q1-Q3, 43.2-187.4). All flaps survived, and no donor site complications were reported. Perioperative complications included wound infection that required debridement (n = 1), immediate postoperative bleeding requiring surgical intervention (n = 1), intraoperative venous congestion (n = 1), and scleroderma recurrence outside the flap (n = 2). When comparing anterolateral thigh and latissimus dorsi flaps, there was no statistically significant difference in the development of surgical complications, number of surgical revisions per patient [median, 4 (Q1-Q3, 4-7) vs 2.5 (Q1-Q3, 1.3, 3), P = 0.0916], or unplanned surgical interventions [median, 0 (Q1-Q3, 0-1) vs 1 (Q1-Q3, 0-1), P = 0.7228]. The reconstruction was successful in improving range of motion in 6 patients, 5 of which reached full weight-bearing. However, all patients had residual limb deformity, and 4 had remaining extremity length discrepancy. CONCLUSIONS Microsurgical flaps are a good option for functional improvement of severe pediatric linear scleroderma affecting the extremities. However, residual limb deformity is common, and patients are often required to undergo additional surgical revisions.

Children and adolescents with localized scleroderma (LS) are at high risk for extracutaneous-related functional impairment including hemiatrophy, arthropathy, seizures, and vision impairment. Compared with adult-onset LS, pediatric disease has a higher likelihood for poor outcome, with extracutaneous involvement twice as prevalent in linear scleroderma, disease relapses more common, and disease duration more than double. Consensus among pediatric rheumatologists on treating patients at risk for significant morbidity with systemic immunosuppressants has led to major improvements in outcome. This review discusses recent progress in assessment and treatment strategies and in our understanding of key disease pathways.

Linear scleroderma is the most common type of localized scleroderma in children. Lesions rarely involve areas other than the skin, and nervous system involvement is even rare. We reported a case of a 6-year-old girl who was admitted to the hospital with recurrent seizures for 4 weeks. Before that, she had left frontal plaques for more than 1 year. Radiological imaging of the brain showed multiple abnormal lesions and skin biopsy of the plaques indicated scleroderma. After drug therapy, the girl had no recurrence of epilepsy, and no obvious abnormalities were found in the reexamination of neuroimaging. We performed further radiological examination on this patient and reviewed the literatures for this rare case.

OBJECTIVE To study disease course and long-term outcome in children with linear scleroderma (SSc) treated with methotrexate (MTX) since diagnosis. METHODS The present study was retrospective and cross-sectional and included consecutive children with linear SSc who were treated with MTX for >1 year and were followed up for at least 2 years. Disease course was analyzed by the number of relapses and treatment changes. Relapse-free survival was examined by Kaplan-Meier analysis, comparing patients with linear SSc and those with other juvenile localized scleroderma (JLS) disease subtypes. Disease activity and damage were assessed by the Localized Scleroderma Cutaneous Assessment Tool and thermography. RESULTS Fifty patients with a mean follow-up duration of 7.8 years and a mean MTX treatment duration of 3.1 years were included. Sixteen percent of patients did not respond to the first course of MTX, and 16% had at least 1 flare. Complete remission was observed in 18.2% of patients who were followed up for 2-5 years, in 80.0% of patients followed up for 10 years, and in 87.5% of patients followed up for >10 years. No significant difference in relapse-free survival between patients with linear SSc and in 17 patients with other JLS disease subtypes was observed. Tissue damage was mild in 42% of patients, moderate in 32%, and severe in 26%. The correlations between severity of tissue damage and linear SSc subtype, disease duration, relapses, and remission were not significant. The relationships between treatment duration and disease relapses (P < 0.05) and severity of tissue damage (P < 0.005) were significant. CONCLUSION Most patients with linear SSc who are treated with MTX achieve complete and long-lasting remission. Overall aesthetic and functional sequelae are moderate, most likely because tissue damage is established early and treatment likely stabilizes the damage. Early diagnosis and MTX treatment, as well as long-term monitoring, are crucial to improve outcome and promptly identify flares.

Scalp linear scleroderma (LSc) is a subtype of localized scleroderma which typically affects young patients and which can be severely disfiguring. Traditional treatment options include bone grafting or tissue expansion. In this report, we present the case of a patient with scalp LSc successfully treated with scar release, autologous fat grafting, and negative-pressure wound therapy (NPWT). A 55-year-old female, with a history of craniectomy for a benign sellar tumor 10 years previously, developed LSc over the frontal scalp with exposure of titanium plates and screws. She was treated with removal of metalwork, scar release, autologous fat grafting from the abdominal wall and immediate application of NPWT. At 3-month postoperative follow-up, the appearance of the depressed lesion and of its margins had significantly improved. Our experience suggests that the combination of autologous fat grafting and NPWT is an effective treatment modality for scalp LSc.

Introducción: El Síndrome de Parry-Romberg es un trastorno neurocutáneo, caracterizado por hemiatrofia facial progresiva, su primer signo clínico es la asimetría facial, se presenta frecuentemente de manera simultánea con esclerodermia lineal. Presentación de caso: Femenina de 54 años de edad, con antecedente de hemiatrofia facial y alteración oftalmológica izquierda desde los 14 años. Discusión: El Síndrome de Parry-Romberg inicia entre las primeras décadas de la vida con hiperpigmentación, atrofia de la dermis, grasa subcutánea, músculo y huesos subyacentes. La atrofia afecta la hemicara siguiendo la distribución del nervio trigémino y sus ramas; este caso coincidió con la edad de inicio, la lesión se limitó a la hemicara izquierda. Conclusión: Debe tenerse presente como diagnóstico diferencial en pacientes con alteración en la simetría facial de larga evolución sin causa aparente, el diagnóstico temprano evita las complicaciones graves, se recomienda un manejo multidisciplinario para mejorar la calidad de vida de los pacientes.

Morphea en coup de sabre is a type of linear localized scleroderma, characterized by a sclerotic skin band in the forehaead and frontoparietal scalp area. It can cause cicatricial alopecia, when affecting the scalp. The disease is mostly unilateral and may be associated with neurological complications and eye defects. Herein, we present a case of bilateral morphea en coup de sabre in a young man, which developed following trauma to the head in childhood 10 years earlier. It produced linear bands of alopecia over the scalp and right eyebrow. The lesions progressed over 4 years, then remained quiescent for 6 years and then restarted progressing again. The patient also complained of repeated frontal headaches but the neurological and ocular examination was normal. Dermoscopy and histopathology were used to confirm the diagnosis. The case is unique because of the bilateral manifestation, preceding trauma and recurrence of disease activity.

Scleroderma is a rare connective tissue disorder categorized into systemic sclerosis and localized scleroderma, also called morphea. Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. We describe a case of linear scleroderma en coup de sabre in a 28-year-old female presenting with seizures and characteristic cutaneous lesions. Imaging findings over a course of 7 years demonstrated waxing and waning signal changes. MR perfusion and spectroscopic imaging, demonstrating decreased cerebral blood volume, increased mean transit time, and decreased metabolites, was performed during a time of progressing radiological and clinical findings. Comparison with other reports in the literature supported several clinical and imaging findings that while not pathognomic, highly suggest the diagnosis of linear scleroderma en coup de sabre. Hyperintense signal on T2W magentic resonance imaging and contrast enhancement on computed tomography and magnetic resonance imaging have been the most commonly described imaging findings. To our knowledge, no previous description of spectroscopic or perfusion imaging of linear scleroderma en coup de sabre have been reported. It is our hope that this report may add MRS and magnetic resonance perfusion findings to a growing knowledge of this rare entity.

ObjectivesTo investigate safety and efficacy of MMF in patients with severe or MTX-refractory juvenile localized scleroderma.MethodsConsecutive juvenile localized scleroderma patients undergoing systemic treatment were included in a retrospective longitudinal study. Patients treated with MMF because they were refractory or intolerant to MTX (MMF-group) were compared with responders to MTX (MTX-group). Disease activity was assessed by Localized Scleroderma Cutaneous Assessment Tool and thermography. Disease course was established on the number of relapses and treatment changes. Relapse-free survival was examined by Kaplan–Meier analysis.ResultsMMF and MTX groups included 22 and 47 patients, respectively. No significant difference in demographics, follow-up duration and treatment before diagnosis was observed between groups. The most represented clinical subtypes in the MMF-group were pansclerotic morphea and mixed subtype (P = 0.008 and P = 0.029, respectively), and linear scleroderma of the face in the MTX-group (P = 0.048). MMF was started because of MTX resistance (18 patients), relapse during MTX tapering/withdrawal (3 patients) and anaphylaxis to MTX (1 patient). After mean 9.4 years of follow-up, 90.9% of patients on MMF and 100% of those on MTX had inactive disease. No significant difference in relapse-free survival between the groups was found (P = 0.066, log-rank test), although MMF likely induced more persistent remission. MMF was well tolerated and combination of MMF and MTX did not increase its efficacy.ConclusionThe present study adds strong evidence on the efficacy and tolerance of MMF in severe and/or MTX-refractory juvenile localized scleroderma. Further controlled studies are needed to prove its efficacy as first line treatment.

BACKGROUND Linear scleroderma "en coups de sabre" is a disease that causes scar-like lesions in the forehead and the scalp, and atrophy of the underlying structures. The result is an acute facial asymmetry that can be distressing to affected young adults. Several surgical treatments are available such as free tissue transfer and synthetic fillers. AIMS In this report, we present a rare case of linear scleroderma "en coups de sabre," which was successfully managed with single-stage autologous fat grafting. PATIENTS/METHODS The patient was a 17-year-old male who presented with a soft-tissue defect in the left forehead region. Treatment consisted of transferring autologous fat into the defect in a retrograde fashion, as described by Coleman, and overcorrecting the defect to account for fat resorption. RESULTS At 6 mo postoperatively, the patient had maintained a satisfactory correction of his left forehead and scalp regions with minimal resorption of fat. CONCLUSION Autologous fat grafting provides a safe and easy approach for the treatment of linear scleroderma "en coups de sabre". With minimal manipulation of the aspirated fat, combined with overcorrection of the defect, long-term clinically satisfactory results can be obtained.

Background: Linear morphoea is a severe morphoea subtype associated with extracutaneous manifestations, potentially permanent disfigurement and functional impairment. Linear morphoea is more prevalent in paediatric patients, and knowledge of disease in adults is limited. The objective of this study was to compare paediatric- and adult-onset linear morphoea, in an exclusively adult population. Methodology: This was a retrospective cohort study of adult patients with linear morphoea seen over a 3-year period at a single-site adult tertiary-referral Connective Tissue Disease centre. Clinical markers of disease severity and course, including anatomical distribution, extracutaneous manifestations, cutaneous symptoms, associated autoimmunity, inflammatory blood parameters, Dermatology Life Quality Index scores, treatment requirements and modified Localised Scleroderma Activity Tool were assessed and compared in paediatric- and adult-onset linear morphoea. Results: Of 298 patients with morphoea seen during the study period, 135 had linear morphoea and 133 were included in the study. Most were female (78.9%), the mean age was 36.5 years and almost half (43.6%) had adult-onset disease. Disease was similarly severe between groups with regard to anatomical distribution, cutaneous symptoms (n = 89, 66.9%), extracutaneous manifestations (n = 76, 57.1%), antinuclear antibody–positivity (n = 40, 40.4%), raised erythrocyte sedimentation rate (n = 27, 25.0%) and associated autoimmune diagnoses (n = 15, 11.3%). Prescribed treatments were similar between groups; 73.7% receiving methotrexate and almost one-third (32.3%) requiring more than one steroid-sparing agent. Those with paediatric-onset had more disease-related damage, with a mean modified Localised Scleroderma Skin Damage Index score of 19.5 (95% confidence interval: 17.0–22.0) versus 8.1 (95% confidence interval: 4.4–11.8; p Conclusion: Linear morphoea in adults can be a severe disease with extracutaneous, autoimmune and systemic features. Adults with paediatric-onset disease appear to have more severe cumulative damage, greater functional impairment and ongoing disease activity. This patient subgroup may require particularly close monitoring and more aggressive therapy.

The localized scleroderma (morphea) is the clinical option of the juvenile scleroderma, the third in prevalence rheumatic condition in pediatrics. The article summarizes all the data on the classification, diagnostics, and differential diagnosis of juvenile localized scleroderma. The recent international guidelines on the localized scleroderma in pediatrics (the European consensus of pediatric rheumatologists, the German and Japanese national guidelines) are presented in the article.

Ultraviolet (UV)A1 phototherapy is effective for T-cell-mediated skin diseases such as atopic dermatitis and mast cell-mediated skin diseases such as mastocytoma. UVA1 phototherapy is also effective against the sclerotic lesions of systemic sclerosis and morphea. Currently, in Japan, access to UVA1 phototherapy is limited because the UVA1 phototherapy device has not yet been approved. On the basis of our experience, we report three patients with localized scleroderma who responded successfully to UVA1 phototherapy. Efficacy was assessed by histological analysis and elastography. UVA1 successfully ameliorated sclerotic lesions, including morphea, linear scleroderma and morphea lesions in a patient with limited cutaneous systemic sclerosis. No side-effects were observed during UVA1 phototherapy.

Lichen nitidus and morphea are common diseases, but an associated localization of both lesions is rare. Here, we describe the first case of lesions distributed along Blaschko's lines. A 24-year-old Japanese woman was referred to our clinic for evaluation of band-like plaques of 18-months history on the right lateral side of her abdomen. In addition, multiple milky-white papules were seen within the plaques. Histopathological examination showed there was sclerosis in the lower half of the dermis and well-circumscribed, dense, papillary dermal lymphohistiocytic aggregations showing a so-called "claw clutching a ball." Immunohistochemical analysis revealed that the morphea and lichen nitidus had similar characteristics. We speculated that unique immunologic events led to the development of lichen nitidus and morphea in our patient.

Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series-supported by expert opinions. The aim of this article was to systematically analyze current data related to the treatment of localized scleroderma en coup de sabre. The databases Scopus, PubMed, and EBSCO were searched for all reports discussing the treatment of localized scleroderma en coup de sabre. The keywords en coup de sabre, “facial linear scleroderma”, and “morphea linearis”, combined with “treatment” or “therapy” were used as search terms. A total of 34 articles analyzed treatment outcomes for patients with localized scleroderma en coup de sabre including 4 retrospective cohort studies, 2 prospective cohort studies, 4 case series, and 24 case reports, representing a total of 69 patients (38 children and 31 adults). Methotrexate was the most commonly investigated treatment (26 patients) with a highest response rate (26/26, 100%). Other treatments included systemic glucocorticosteroids (nine patients), followed by UVA1 (four patients), mycophenolate mofetil (two patients), hydroxychloroquine (five patients), abatacept (two patients), tocilizumab (three patients), cyclosporine (one patient), interferon gamma (one patient), PUVA therapy (two patients), NB-UVB therapy (one patient), and pulsed dye laser (one patient). Reconstructive and surgery treatment was successfully used for lesions with settled disease activity to improve the cosmetic aspect of the lesions. Conclusion: methotrexate is the most often-studied treatment and reported good clinical outcomes in children and adults with localized scleroderma en coup de sabre.

Autologous fat is a biological substance that attracting increased scientific interest. Autologous fat considered as ideal filler due to its biocompatibility without risk of an allergic reaction or rejection. Likewise, this substance could be obtained easily, and costs are relatively low. Therapeutic indications for use fat grafting appear day by day, as it is recognized as an effective, reliable methodology and enhancing of areas and pathologies of application in medical specialties. The analysis results show that autologous fat transplantation gives a possibility to compensate for aesthetic and functional signs caused by facial scleroderma.

Linear scleroderma is a variant of localized scleroderma. We report a 43-year-old woman who had developed left arm weakness and linear scleroderma on her back during pregnancy at 25 years of age, followed by left hemifacial atrophy and left leg weakness. She had multiple linear scleroderma lesions on her trunk and left limbs, left eyelid ptosis, impairment of vertical movement and abduction of the left eye, left hemifacial atrophy, and weakness and atrophy of the sternocleidomastoid, trapezius, and proximal limb muscles on the left side. On serology, antibodies to U1-ribonucleoprotein and Jo-1 were positive; anti-scleroderma-70 antibody was negative. Skin biopsy demonstrated increased hypertrophic collagen fibers without inflammatory infiltrates. Needle electromyography of left limb muscles revealed mild neurogenic patterns; left quadriceps muscle biopsy showed chronic neurogenic changes. Brain magnetic resonance imaging revealed mild left hemispheric atrophy. This is a rare case of linear scleroderma and Parry-Romberg syndrome presenting with widespread ipsilateral neurogenic manifestations.

Scleroderma, "en coup de sabre" is a rare disorder, characterized by linear depressed scarring at frontoparietal area of the face, seldom associated with ophthalmological findings. Extraocular muscle involvement and paralytic strabismus are rarely associated with linear scleroderma. Here we are reporting a case of unilateral linear scleroderma in an 8-year-old child, with features of strabismus fixus secondary to fibrosed medial rectus (MR) muscle, enophthalmos and en coup de saber (sword-like scar) on the eyebrow. MRI orbit was suggestive of the bulky MR and the inferior rectus muscle. MR recession along with partial loop myopexy resulted in cosmetically acceptable ocular alignment in this case. Histopathology of the portion of muscle and intermuscular septum showed diffuse fibrosis of the muscle. Linear scleroderma may be the causative etiology of strabismus fixus in our case, never reported before in literature.

En coup de sabre is an unusual variant of localized scleroderma characterized by its distinct location involving the frontoparietal region of the forehead and scalp. The authors describe a rare case of en coup de sabre in a 3-year-old boy whose disease onset was at 12 months of age. This article presents the clinical manifestations of continuous changes in the lesion photographed over time. The clinical presentation, laboratory results, and radiological findings together with a brief discussion of the management of the disease are discussed.

Background The aim of this study was to: (i) describe the abnormalities seen on brain imaging in a group of children with en coup de sabre (EDCS) with/without Parry-Romberg syndrome (PRS); and (ii) identify clinical predictors of brain imaging abnormalities. Methods This was a single centre (Great Ormond Street Hospital, London) retrospective case series of patients with ECDS/PRS seen from 2000 to 2018. We identified patients with cutaneous manifestations consistent with the clinical descriptions of ECDS/PRS. Presenting clinical, laboratory, and radiological brain findings are described. Results are expressed as medians and ranges or frequencies and percentages. Fisher’s exact test was used to identify clinical associations with magnetic resonance imaging (MRI) abnormalities. Results Fourteen patients were studied: 6 males and 8 females; median age 14 years (range 3–20). We observed neuroimaging abnormalities in 2/6 ECDS and 5/8 ECDS/PRS patients. White matter signal abnormality, dystrophic calcification, leptomeningeal enhancement, and sulcal crowding were the typical findings on brain imaging. A total of 50% of patients had no MRI abnormality despite some of these patients having neurological symptoms. The presence of seizures was significantly associated with ipsilateral enhanced white matter signalling on MRI (p Conclusions In summary, we observed several distinct radiographic patterns associated with ECDS/PRS. Seizure disorder was strongly associated with the presence of ipsilateral enhanced white matter signalling. Improved neuroimaging techniques that combine morphological with functional imaging may improve the detection rate of brain involvement in children with ECDS/PRS in the future.

The en coup de sabre variant of linear scleroderma typically occurs in children. We report a unique case of adult-onset en coup de sabre scleroderma in a patient with linear localized scleroderma profunda. The patient was treated with oral steroids and oral methotrexate improving her cutaneous disease. This case highlights the importance of a thorough cutaneous examination as this adult patient developed an entity traditionally believed to occur in childhood.

Background: Linear atrophoderma of Moulin (LAM) is a dermatosis that affects children and adolescents characterized by hyperpigmented and atrophic linear lesions following Blaschko lines. So far, less than 50 cases have been published. Therefore, it is a rare entity with unknown etiology and a chronic and self-limited course. Histologically, it is described as hyperpigmentation of the basal layer without the involvement of the dermis and subcutaneous tissue. No specific treatment exists currently. Localized scleroderma is a chronic and progressive autoimmune connective tissue disorder that affects the skin and adjacent tissues, characterized by abnormal collagen deposition and alteration in elastic fibers, blood vessels, and annexes. No reports have been published on the coexistence of localized scleroderma and LAM. Case report: We describe the case of an 11-year-old male with a clinical diagnosis of linear scleroderma since 5 years of age. Four years later, the patient developed atrophic and hyperpigmented lesions following Blaschko lines in the posterior trunk. A biopsy of both dermatoses was performed: the trunk showed epidermis with hyperpigmentation of the basal layer, and within the dermis, no alteration in the collagen bundles; the forearm biopsy corroborated scleroderma. Based on the clinical-pathological correlation, LAM coinciding with localized linear scleroderma was diagnosed. Conclusions: LAM is an infrequent entity by itself. Moreover, its coexistence with sclerodermiform syndrome has not been reported in the indexed literature.

The autoimmune basis of segmental vitiligo (SV) has only recently been recognized. Systemic autoimmune diseases are less frequently associated compared to non-segmental vitiligo (NSV), but localized skin disorders – in particular linear morphea – have been repeatedly observed in patients with SV. The inflammatory response is documented on a clinical level with cases displaying erythematous borders or a hypochromic stage, on a histopathological level with predominantly CD8 lymphocytes migrating toward the basal layer and by flow cytometry demonstrating the antimelanocyte specificity of these cytotoxic T cells. The increased risk for halo naevi and NSV in these patients further underline the immune-mediated mechanisms of SV. Nonetheless, the localized and unique distribution pattern points to somatic mosaicism. This places SV in a category of similar diseases such as lichen striatus, blaschkitis, linear lupus erythematosus, and linear scleroderma where an immune reaction against genetically mutated skin cells is believed to be the underlying cause. All these disorders are characterized by a young age of onset, a temporary disease activity with spontaneous resolution, limited response to treatment, and often long-term sequelae. Although challenging, genetic research proving this genetic mosaicism could offer crucial insights into the pathogenesis of both segmental and non-segmental vitiligo.

Treatment of scleroderma in children is challenging since little is known about its pathogenesis. Herein, we review the most recent evidence regarding the treatment of juvenile scleroderma. According to the recent recommendations for Pediatric Rheumatology in Europe (SHARE), systemic treatment in localized scleroderma is needed when there is a risk for disability, such as in generalized or pansclerotic morphea and progressive linear scleroderma. In juvenile systemic sclerosis, the introduction of the severity score, J4S, has standardized the assessment of the patients in the daily practice and allowed a more tailored therapeutic approach. Since, to date, no clinical trial is available in JSSc, due to its rarity, the treatment is based on adults’ experience. The recent recommendations for juvenile scleroderma represent an important instrument to standardize the treatment approach, confirm the role of methotrexate, and open new windows for effective experimental treatments, such as mycophenolate mofetil and biological agents, for severe or refractory cases.

En coup de sabre morphea is a clinical variant of morphea, presenting as a linear depressed, atrophic area in the paramedian forehead or the frontoparietal scalp, resembling a stroke from a sword. It affects the skin and subcutaneous connective tissues, with possible extension to the underlying musculature, cartilage, and bone and variable association with neurologic symptoms. A 50-year-old woman presented to our clinic for evaluation of an atrophic lesion on her forehead and scalp appearing 1 year before, progressively extending over time. An alopecic atrophic area involving the skin and subcutaneous tissues of her right forehead and scalp arranged in a linear fashion with an “en coup de sabre” appearance was observed in relation with hair loss in the affected area. Reflectance confocal microscopy showed marked hyperreflective areas with severe eccrine gland atrophy. All sebaceous glands had disappeared, with sporadic follicular persistence reduction. Histopathological examination of a punch biopsy specimen taken from a central parietal alopecic area was consistent with a diagnosis of morphea. To our knowledge, this is the first report regarding the use of reflectance confocal microscopy as an ancillary diagnostic technique in linear localized morphea of the scalp and face. This noninvasive technique may represent a useful tool in distinguishing between early stages of the disease, with prevalence of inflammatory lymphocytic infiltrate, and late stages characterized by more prominent sclerosis with mild or absent signs of inflammation.

Silent sinus syndrome (SSS) is the spontaneous unilateral collapse of the maxillary sinus and orbital floor with complete or partial opacification of the collapsed sinus. The key features in a patient who presents with SSS are painless progressive unilateral maxillary sinus disease in the absence of rhinosinusitis, trauma, or surgery. SSS is a rare disorder but could be under-diagnosed because of a lack of recognition. SSS is characterized by spontaneous and progressive enophthalmos (“sunken” eye-eye recession into the globe) and hypoglobus (globe displaced downward; and a drop in the pupillary level), so it is common for these patients to present first to an ophthalmologist. Although mostly observed in adults, there have been reports of SSS in children. SSS in younger individuals has characteristic clinical and radiologic signs with, in many cases, abnormal intranasal anatomic characteristics on the affected side. SSS should be differentiated from other causes of spontaneous enophthalmos, such as Parry-Romberg syndrome and linear scleroderma. The aim of this report was to alert the reader to the imaging findings in patients with SSS. This syndrome is well recognized by rhinologists and to a lesser extent to ophthalmologists but remains relatively unknown to general radiologists.

Linear scleroderma en coup de sabre (LSCS) is a clinical variant of morphea that presents with a linear alopecic patch over the frontal scalp. Linear alopecia areata may present as a close differential in the incipient stages of LSCS in the pediatric population, especially when the binding down and pigmentary changes characteristic of LSCS have not developed. Histopathology may also be noncontributory in such scenarios. We hereby report a case of linear alopecia in a 5-year-old Indian girl and highlight the role of trichoscopic evaluation in clinching the diagnosis. This emphasizes the importance of clinicodermoscopic-pathological correlation over clinicopathological correlation for cutaneous diagnosis.

Parry Romberg disease (hemifacial atrophy) and linear scleroderma (coup de sabre) are progressive, usually unilateral facial atrophies of unknown cause. The gold standard treatment for these patients has been microsurgical reconstruction following the "burning out" of the facial atrophy and stable contour for 2 years.The authors report their experience treating patients with hemifacial atrophy and linear scleroderma with free tissue transfers between 1989 and 2016. A modified parascapular flap based on the circumflex scapular artery was most commonly used.A total of 177 patients were included. The most common complication was hematoma, occurring in 12 patients (7 percent). Follow-up ranged from 1 to 26 years. All patients subjectively experienced improved facial symmetry and aesthetics. No disease process has recurred to date, even in cases of severe, progressive disease.In the authors' experience, patients treated early in their disease course have immediate and sustained correction of their deformity, with slowing or in most cases cessation of the disease process following free tissue transfer. The authors now advocate for immediate reconstruction for active disease, especially in young children.Therapeutic, IV.

Objective.To identify clinical features that define disease activity in pediatric localized scleroderma (LS), and determine their specificity and importance.Methods.We conducted a multicenter prospective study of patients with active and inactive LS skin lesions. A standardized evaluation of a single designated study lesion per subject was performed at 3 visits. We evaluated the pattern and correlation between assessed features and physician’s global assessments of activity (PGA-A).Results.Ninety of 103 subjects had evaluable data; 66 had active and 24 inactive disease. Subjects had similar age of onset, sex, and disease patterns. Linear scleroderma was the most common subtype. Features specific for active disease included erythema, violaceous color, tactile warmth, abnormal skin texture, and disease extension. Scores for these variables changed over time and correlated with PGA-A of the lesion. Active and inactive lesions could not be distinguished by the presence or level of skin thickening, either of lesion edge or center. However, in active lesions, skin thickening scores did correlate with PGA–A scores. Regression analysis identified the combination of erythema, disease extension, violaceous color, skin thickening, and abnormal texture as predictive of PGA-A at study entry. Damage features were common irrespective of activity status.Conclusion.We identified variables strongly associated with disease activity, expanding upon those used in current measures, and determined their relative importance in physician activity scoring. Skin thickening was found to lack specificity for disease activity. These results will help guide development of a sensitive, responsive activity tool to improve care of patients with LS.

Linear scleroderma en coup de sabre with ophthalmic findings has been previously described in the literature on numerous occasions. A 57-year-old woman presented with focal trichiasis secondary to tarsal thinning, adjacent to a linear brow and forehead deformity consistent with linear scleroderma en coup de sabre. Cases of linear scleroderma en coup de sabre involving the eyelids have been reported, most often with madarosis, ptosis, or skin atrophy; however, to the authors' knowledge, this is the first reported case of linear scleroderma associated with trichiasis and involvement of the deeper eyelid tissues, particularly the tarsus.

Linear scleroderma “en coup de sabre” is a subset of localized scleroderma with band-like sclerotic lesions typically involving the frontoparietal regions of the scalp. En coup de sabre and Parry–Romberg syndrome are variants of linear morphea on the head and neck that can be associated with neurologic manifestations. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. We present a case of an 8-year-old girl with a left frontoparietal “en coup de sabre” scalp lesion and describe the neuroimaging findings of frontoparietal white matter lesion discovered incidentally on routine magnetic resonance imaging. The patient had no neurologic symptoms given the lesion identified. Keywords: en coup de sabre, MRI

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, bosentan, 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.

‘En coup de sabre’ or morphea, is a linear scleroderma that typically affects the frontoparietal region of patients resulting in a band-like scar that looks like a sword cut across the face. This case report describes a 13-year-old male patient with a history of ‘en coup de sabre’ referred to a dental department because of facial and dental involvement. To the authors' knowledge this has only previously been described on one occasion. The clinical presentation, clinical findings and a brief discussion of management, including the complicating effects of ‘en coup de sabre’ are discussed. CPD/Clinical Relevance: By describing the facial and dental features of this rare condition to the dental community, the authors wish to increase awareness of the condition.

Background Localized scleroderma is characterized by a thickening of the skin from excessive collagen deposits. It is not a fatal disease, but quality of life can be adversely affected due to changes in skin appearance, joint contractures, and, rarely, serious deformities of the face and extremities. We present six cases of localized scleroderma in face from our surgical practice. Methods We reviewed six localized scleroderma cases that were initially treated with medication and then received follow-up surgery between April 2003 and February 2015. Six patients had facial lesions. These cases presented with linear dermal sclerosis on the forehead, oval subcutaneous and dermal depression in the cheek. Results En coup de sabre (n=4), and oval-shaped lesion of the face (n=2) were successfully treated. Surgical methods included resection with or without Z-plasty (n=3), fat graft (n=1), dermofat graft (n=1), and adipofascial free flap (n=1). Deformities of the affected parts were surgically corrected without reoccurrence. Conclusion We retrospectively reviewed six cases of localized scleroderma that were successfully treated with surgery. And we propose an algorithm for selecting the best surgical approach for individual localized scleroderma cases. Although our cases were limited in number and long-term follow-up will be necessary, we suggest that surgical management should be considered as an option for treating scleroderma patients.

Linear scleroderma is a characteristic form of scleroderma that typically affects children. Ocular manifestations may be present, especially when the frontoparietal area of the head is affected. We present the case of a 5-year-old boy with craniofacial linear scleroderma ("en coup de sabre") who developed exudative retinal detachment. Angiographic and neuroimaging findings are presented, and the importance of regular fundus examination is highlighted.

Linear scleroderma is a rare form of localized scleroderma. The involvement of the frontal or frontoparietal region with linear scleroderma is called en coup de sabre (ECDS). ECDS presents as linear sclerotic depressed groove appearing on the frontoparietal region. It is usually unilateral and extends from the forehead into the frontal scalp. Its paramedian location is more common than its median one. The sclerotic changes affect the soft tissue and muscles as well as adjacent structures.1 Linear scleroderma has diverse etiologies, and its pathogenic mechanism is still unclear. Botulinum toxin (BTX) is an exotoxin produced from Clostridium botulinum. It blocks the release of acetylcholine from the cholinergic nerve end plates, resulting in inactivity of the muscles or glands innervated. It has gained a great interest in cosmetic dermatology for its effects on hyperkinetic facial lines and focal hyperhidrosis.2 Recently, BTX has also been used experimentally in many noncosmetic dermatologic conditions with agreeable results.2 We present 2 patients with disfigurement-associated ECDS treated successfully with BTX injection.

Linear scleroderma is a form of localized scleroderma that presents as band-like sclerotic lesions. When it occurs on the scalp or forehead, it is called "en coup de sabre scleroderma" (ECDS). Extracutaneous associations, mostly neurologic, have been described with ECDS. We report a patient with ECDS associated with multiple cerebral cavernomas. This serves to highlight the importance of neuroimaging and multidisciplinary team involvement in patients with ECDS.

Melorheostosis (MEL) is a rare benign bone disorder that can be associated with several anomalies, including vascular abnormalities, nevus sebaceus, unilateral nevoid telangiectasia, linear scleroderma and hypertrichosis. We report the case of a 6-year-old patient who showed an unusual co-occurrence of bone hyperostosis and different skin lesions affecting the same side of the body: MEL, verrucous epidermal nevus, connective tissue nevus, linear scleroderma-like disorder, hyperpigmentation and hypertrichosis. The spatial co-occurrence of these conditions made us speculate as to whether they originated from a common genetic mechanism or if their co-occurrence was completely accidental.

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. We searched databases including PubMed, Elsevier and MedLine and Wanfang, reviewing publications from 2013 to 2019. Selected earlier publications were also reviewed. 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. 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.

Linear scleroderma is the most common subtype of localized scleroderma (LoS) in children. It can be associated with extracutaneous manifestations and long-term sequelae. Thus, appropriate diagnosis and management are key to improve the prognosis. In this review, we summarize the most relevant recent publications for the diagnosis, evaluation of disease activity and adequate management of patients with linear scleroderma.There are specific clinical features that indicate activity in LoS; dermoscopy and Wood's lamp may be useful. Summarizing, scoring methods seem to provide the most adequate assessment of LoS; but several biomarkers that correlate with activity have been studied: E-selectin and IL-2 receptor, CD34+ dermal dendritic cells and Th/Th1 immunophenotype with decreased T helper (Th2), T regulatory (Tregs), B and natural killer (NK) cells. Recent studies propose hydroxychloroquine monotherapy and tocilizumab as potential therapeutic options.Clinical evaluation, both physical exam and history, is the most important aspect in diagnosing and assessing activity of linear scleroderma. Clinical scoring methods may be most useful for evaluation of activity; eventually, other biomarkers could be relevant in clinical practice. For most patients with linear scleroderma, the first choice of treatment is methotrexate, but physical therapy, plastic surgery and/or orthopedic management are key to improve residual limitations and quality of life. VIDEO ABSTRACT: http://links.lww.com/MOP/A35.

Retinal vasculitis is a sight-threatening condition that can occur as an isolated ocular disorder or in association with a number of systemic diseases. Parry-Romberg syndrome, also known as progressive hemifacial atrophy (PHA), is a rare disorder of unknown etiology characterized by unilateral facial atrophy and is associated with multiple ophthalmologic and neurologic manifestations. Here we report the case of a 17-year-old man with no prior diagnosis of PHA, who presented with a sudden onset of floaters and decreased vision in the right eye; he was found to have retinal vasculitis and uveitis in the right eye. Routine workup did not reveal the cause of retinal vasculitis. However, thorough physical examination demonstrated features of PHA overlapping with linear scleroderma en coup de sabre. The patient was started on treatment with systemic steroids with a later addition of methotrexate; he responded to treatment with considerable improvement in his symptoms and ophthalmologic examination.

Linear scleroderma “en coup de sabre” (LSES) variant is a cephalic subtype of localized scleroderma that can be associated with extracutaneous stigmata, such as epilepsy, dementia syndromes, as well as focal central nervous system neurologic deficits. While the pathophysiology of cutaneous linear scleroderma includes endothelial cell injury and up regulation of pro-fibrogenic pathways, the basis of LSES-associated neurologic complications is largely unknown. We report a patient with a history of LSES who developed intractable epilepsy and cognitive decline. Magnetic resonance imaging (MRI) of the brain exhibited numerous persistently enhancing brain lesions. Due to progressive neurologic deterioration over a period of 7 years, despite interventional therapy, a brain biopsy was performed. Neuropathologic analysis exhibited acute and chronic cortical ischemia associated with a small vessel lymphocytic vasculitis. Direct immunofluorescent studies showed C5b-9 and IgG deposition on endothelium while indirect immunofluorescent studies demonstrated reactivity of the patient’s serum with the microvasculature of the patient’s own brain tissue and generic human umbilical vein endothelial cells indicative of anti-endothelial cell antibodies. Therapy focusing on damaged endothelium was implemented. The interleukin-6 (IL-6) receptor inhibitor tocilizumab was used and the patient improved dramatically, likely reflecting the drug’s effect on the replenishment of endothelial progenitor cells.

Background Parry–Romberg syndrome, also known as progressive hemifacial atrophy, is a rare degenerative disorder with numerous distinctive clinical presentations. It is usually slowly progressive, occurring more in females, and affects primarily one side of the face; it causes unilateral atrophy and loss of skin, subcutaneous tissue, muscles, and bones, and can even extend to oral structures. Other involvements that can occur are ocular and neurological; however, it is frequently associated with linear scleroderma, known as en coup de sabre. The etiology of the disorder is unknown, although some consider it a variant of morphea (localized scleroderma) and others proposed a malfunction of the sympathetic system as a cause. Imaging studies can support diagnosis and reveal the extent of the disease. Moreover, with the wide systemic involvement in such a condition, a multidisciplinary approach is crucial. Case presentation A 35-year-old Dinka woman presented with left hemifacial atrophy associated with left-sided body hemihypoesthesia and glaucoma with overlapping linear scleroderma “en coup de sabre” for 5 years. Conclusions Parry–Romberg syndrome is a very rare entity causing progressive hemifacial atrophy that could also be associated with linear scleroderma. It has devastating outcomes due to its various systemic involvements; therefore, a multidisciplinary approach is required together with further studies to be performed in order to identify the key etiology and construct a clear guideline for management.