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Linear scleroderma is a localized variety of scleroderma characterized by fibrotic areas of the dermis involving head region without systemic features. Ocular involvement has been sparsely reported in the form of episcleritis, dry eye, and uveitis. We describe a 42-year-old man with linear scleroderma, en coup de sabre type with associated nasolacrimal duct obstruction and prolonged dacryocystitis.

We present the case of a 58-year-old female presenting with acute onset headache and decrease in left-sided facial sensation. The patient's background included diabetes Type 2, hypertension, migraine, anxiety with depression and scleroderma in her childhood. Imaging revealed foci of right frontal calcification and confluent white matter changes, reported as sequelae of a previous ischaemic episode. Following a second presentation with the same symptoms, further imaging showed a linear soft tissue scar overlying these changes, which suggested a diagnosis of linear scleroderma (en coup de sabre). On questioning, it was found that this had developed during the patient's late teens but had not progressed since that time. Coup de sabre type linear scleroderma is often associated with intracranial imaging findings, even in the absence of symptoms. The pathogenesis of neurological symptoms is poorly understood but does seem to respond to immunosuppression.

Linear scleroderma is a kind of disease that can cause complication of the central nervous system. Sometimes, ipsilateral intracerebral or white matter lesions in the brain magnetic resonance imaging are noted. Nystagmus is important for the differential diagnosis of dizziness. Positional nystagmus was classified into regular direction nystagmus, direction changing positional nystagmus (DCPN) and irregular nystagmus by their character. DCPN is defined as a nystagmus that changes its direction with different head and body positions, and it can be the sign of lesion in the central vestibular system. Recently, we experienced a 17-year-old woman who had a scalp linear scleroderma, treated for about 10 years and showed DCPN induced by positional nystagmus test. We report a case of linear scleroderma accompanied by cerebellar lesion with a brief review of literature.

We present an interesting case of a patient with linear scleroderma of the ‘en coup de sabre’ type who was treated with an upper fixed appliance to align a maxillary canine. It started out as a routine straightforward case of aligning an impacted canine using a conventional technique but developed into a serious orthodontic dilemma. Clinical Relevance: The atypical response to treatment suggests a possible link between the connective tissue disorder and the orthodontic complications this case developed.

En coup de sabre (linear scleroderma of face) is a rare type of morphea (localized scleroderma) involving frontoparietal area of the forehead and scalp. Many triggering factors have been implicated in the development of morphea like trauma, immobilization, bacille Calmette–Guerin (BCG) vaccination, injections of vitamin K, mechanical compression from clothing, etc. Linear scleroderma primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. In this article, we describe a case of 26 year old female who presented with a three months history of brownish indurated plaque of skin on the frontal and forehead regions of the head. The patient gave a history of trauma at the same site six years back. The diagnosis of morphea was made clinically supported by histopathological features of the skin biopsy. Her neurological examination was normal. ANA was negative. Brain MRI didn’t reveal any abnormality. She was treated with topical tacrolimus 0.1% ointment. The late onset en coup de sabre is a rare presentation and hence reported.

Scleroderma is a chronic disease of unknown aetiology, consisting of two distinct clinical entities: systemic sclerosis (SSc) and localised scleroderma (LSc or morphea) [1]. SSc affects the skin and various internal organs, whereas LSc exclusively involves the skin and its underlying tissues. Consistent with a difference in disease process, SSc/LSc overlap is relatively rare [2, 3]. We report herein a unique case of SSc complicated with LSc following fracture surgery.A 69-year-old Japanese woman [...]

The aim of this study is to objectively evaluate whether linear scleroderma (LS) follows Blaschko’s lines (BL) in Korean patients using digital image processing. Thirty-two patients with LS were examined. According to the patients’ clinical photographs, their skin lesions were plotted on the head and body charts. With the aid of graphics software, a digital image was produced that included an overlay of all the individual lesions and was used to compare the graphics with the published BL. To investigate the image similarity between the graphic patterns of the LS and BL, each case was analyzed by means of Hough transformations and Czekanowski’s methods. The comparative investigation of the graphic similarity of distributional patterns between the LS and BL showed that Czekanowski’s similarity index was 0.947 on average. In conclusion, our objective results suggest that the graphic patterns of the distribution of the LS skin lesions showed a high degree of similarity and in fact were almost identical to that of BL which may be the lines of embryonic development of the skin. This finding may suggest that some developmental factors during the embryological age could constitute the cause of LS.

Purpose To describe a Coats-like response and central nervous system vasculitis and its successful management in a patient presenting with en coup de sabre scleroderma. Methods Retrospective interventional case report. Chart review. Results A 20-year-old white male patient with en coup de sabre scleroderma on treatment with oral cyclophosphamide and prednisone for associated central nervous system inflammation presented with photopsias in the ipsilateral side of the scleroderma. A magnetic resonance imaging before presentation had shown lesions suggestive of localized central nervous system vasculitis. Ocular examination showed vitritis and multiple venous and arteriolar saccular dilatations with lipid exudation and subretinal fluid. These were treated with intravitreal bevacizumab, focal laser, and subtenon triamcinolone acetonide. After a year of follow-up, there was near resolution of the exudation and complete resolution of the vitreous inflammation, and the patient has been clinically stable with no visual loss. Conclusion Coats-like response with ocular inflammation can be associated with central nervous system vasculitis in patients with linear scleroderma. Careful clinical evaluation and aggressive multipronged treatment are necessary to ensure a successful outcome.

Morphea is a fibrosing disorder of the skin and subcutaneous tissues, wherein the overabundant collagen deposition destroys adnexal structures and hair follicles. In linear morphea erythematous or violaceous patches or plaques are seen with central sclerosis and active red border distributed in linear configuration which over time become sclerotic, white or hypopigmented. We report a series of cases encompassing all the three clinical variants of linear morphea. Case one had en coup de sabre deformity, case two had progressive facial hemiatrophy and case three had linear limb morphea with involvement of the face. This series is being reported for its rarity.

A 17-year-old Caucasian woman presented for evaluation and treatment of a tender expanding linear plaque on her left flank. Biopsy findings were consistent with morphea. Treatment initially included intralesional steroid injections and topical calcipotriene ointment, followed by methotrexate and excimer laser. The lesion decreased in size considerably with relief of symptomatic discomfort by 7 months. An excisional biopsy of a persistent eroded papule on the superior aspect of the morphea plaque revealed dermal thickening and sclerosis with superimposed perforation of a calcified nodule. Localized scleroderma, or morphea, is an autoimmune disease of the skin and underlying subcutaneous tissue primarily affecting the pediatric population. The excimer laser has been reported to effectively treat a variety of dermatologic conditions, including morphea. Its mechanism of action may be via depletion of T cells, altering apoptosis-mediating molecules and decreasing cytokine expression. Methotrexate is also useful for the acute and deep forms of morphea and has been shown to decrease levels of inter leukins-2 and -6, tenascin, and mast cells. This patient had a good clinical response with a combination of these two modalities. The epidermal perforation with transepidermal elimination of calcified necrotic collagen is a unique complication that may have been secondary to this combination treatment modality.

Aim: To present a case of linear scleroderma (LS) involving the extraocular muscles and periorbita with follow-up over an 18-year period. Method: Initial presentation was of a female aged 9 years with progressive left unilateral ptosis which ultimately underwent surgical correction. Results: The main features of ‘en coup de sabre’ subsequently manifested on the left side of the face and head. An unusual feature was the characteristic subcutaneous cleft which also appeared on the opposite side above the right eyebrow. The left eye went on to develop a corneal ulcer with resulting poor visual acuity. An ipsilateral oculomotility disorder was then noted in addition to poor eyelid closure. Conclusion: This case highlights the need to exclude ‘en coup de sabre’ as a potential differential diagnosis in any case of progressive or recurrent ptosis, especially in childhood. As oculomotility may also be affected ultimately, it could lead to corneal compromise following ptosis correction.

Evaluation of: Martini G, Ramanan AV, Falcini F et al.: Successful treatment of severe or methotrexate-resistant juvenile localized scleroderma with mycophenolate mofetil. Rheumatology (Oxford) 48, 1410–1413 (2009). This retrospective study reports the outcome of mycophenolate mofetil (MMF) treatment in ten juvenile patients with severe or methotrexate-resistant localized scleroderma (two with pansclerotic morphea, five with linear scleroderma and three with generalized morphea). The clinical efficacy of MMF was assessed by clinical examination and thermography. The authors demonstrated efficacy of MMF in reducing the signs of inflammation, softening or lightening of skin lesions, which allowed reduction in dosage or withdrawal of corticosteroids and methotrexate in all patients. MMF was well tolerated and the investigators concluded that MMF is a valid alternative to methotrexate in arresting disease progression in juvenile localized scleroderma.

Objective Childhood scleroderma encompasses a rare, poorly understood spectrum of conditions. Our aim was to ascertain the incidence of childhood scleroderma in its different forms in the UK and Ireland, and to describe the age, sex, and ethnicity of the cases. Methods The members of 5 specialist medical associations including pediatricians, dermatologists, and rheumatologists were asked to report all cases of abnormal skin thickening suspected to be localized (including linear) scleroderma or systemic sclerosis (SSc) in children

The patient was a 12-year-old girl with linear scleroderma distributed on the right abdomen, dorsal aspect of the right thigh, lower leg and foot. The initial regimen of oral prednisolone and methotrexate, or i.v. methylprednisolone failed in the treatment of the scleroderma. Then bath psoralen and ultraviolet A therapy (bath-PUVA) therapy of 0.2 J-4.0 J/cm(2) daily to total doses 62.8 J/cm(2) combined with oral prednisolone was started. After bath-PUVA therapy, regression of the skin sclerosis was observed, the possible mobile range of the right ankle was increased and histological examination confirmed improvement of the sclerosis. The successful results of bath-PUVA therapy in this case suggest its utility for localized scleroderma.

The term “en coup de sabre” is used for linear scleroderma located in the temporoparietal region that produces sharp bordered furrowing with alopecia when extending to the scalp. Although it is a benign disease, the resultant marked deformity often necessitates treatment. While in some cases atrophy is confined to the skin and subcutaneous tissue, cases with underlying muscle and bone involvement have also been reported. Symptoms and clinical findings in linear scleroderma of the face show variations. We present a case of “en coup de sabre” linear scleroderma of the cheek affecting only the skin and subcutaneous fat tissue without any accompanying disorder.

We report a 4-year-old girl presenting with progressive linear scleroderma affecting the right leg. Biopsy specimen disclosed typical histopathological findings of localized scleroderma. Right leg magnetic resonance imaging (MRI) showed high signal areas on T(2)-weighted images on the subcutaneous fatty tissue, muscles and bone marrow, suggesting that skin inflammation extended to the bone marrow. Oral corticosteroid therapy was instituted with improvement of both skin sclerosis and MRI findings. Our observations suggest that MRI examination should be considered in patients with localized scleroderma to evaluate the extension of the inflammation., The Journal of dermatology, 37(1), pp.81-84; 2010

Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. The “en coup de sabre” subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. Here, we report one patient with typical linear scleroderma “en coup de sabre” (LSCS). Initially, he presented with refractory partial seizures before the characteristic skins lesion on his head developed. This was a rare case with obvious brain parenchyma involvement. We did not prescribe medication but performed serial brain magnetic resonance imaging follow-up for the intraparenchymal lesion. The atrophic changes of the skin, face and brain remained the same, and his seizures had not worsened at the most recent follow-up. Parry-Romberg syndrome, a very similar condition, should be differentiated from LSCS.

Background Localized scleroderma also called morphea is a skin disorder of undetermined cause. The widely recognized Mayo Clinic Classification identifies 5 main morphea types: plaque, generalized, bullous, linear and deep. Whether each of these distinct types has a particular clinical course or is associated with some patient-related features is still unclear. Methods We report here a retrospective series of patients with localized scleroderma with an attempt to identify features related to the type of lesion involved. The medical records of all patients with a diagnosis of localized scleroderma were reviewed by skilled practitioners. Lesions were classified according to the Mayo Clinic Classification. The relationship between each lesion type and various clinical features was tested by non-parametrical methods. Results The sample of 52 patients included 43 females and 9 males. Median age at onset was 30 y (range 1–76). Frequencies of patients according to morphea types were: plaque morphea 41 (78.8%) (including morphea en plaque 30 (57.7%) and atrophoderma of Pasini–Pierini 11 (21.1%)), linear scleroderma 14 (26.9%). Nine patients (17.3%) had both types of localized scleroderma. Median age at onset was lower in patients with linear scleroderma (8 y (range 3–44)) than in others (36 y (range 1–77)) (p = 0.0003). Head involvement was more common in patients with linear scleroderma (37.5%) than in other subtypes (11.1%) (p = 0.05). Atrophoderma of Pasini–Pierini was never located at the head. Systemic symptoms, antinuclear antibodies and the rheumatic factor were not associated with localized scleroderma types or subtypes. Conclusion These results suggest that morphea types, in adults are not associated with distinct patient features except for age at disease onset (lower) and the localization on the head (more frequent), in patients with lesions of the linear type.

BACKGROUND Facial linear scleroderma results in depressed atrophic scars. Autologous fat transplantation has been widely used, and fat appears to be an ideal material for filling depressed atrophic scars and contour deformities, but long-term results for autologous fat transplantation are controversial. OBJECTIVE To review the short- and long-term results of 20 patients who underwent multiple autologous fat transplantations for depressed atrophic scar correction. METHODS Twenty patients with clinically inactive facial linear scleroderma were included. They received at least two transplantations and had a 12-month follow-up evaluation. RESULTS On the forehead, 51% to 75% improvement (average grading scale: 2.4) was achieved when observed at least 12 months after the last treatment. For the chin, correction was poor (average grading scale: 0.7) with less than 25% improvement. The infraorbital area showed fair correction, but the nose showed poor correction. Two of three patients with scalp reduction surgery showed excellent results, showing only slight scar widening. CONCLUSIONS Autologous fat transplantation is an effective method for long-term correction of depressed atrophic scars left by linear scleroderma on the forehead but is less effective for corrections on the nose, infraorbital area, and chin.

Background: Localized scleroderma (LSc) is characterized by cutaneous fibrosis and various autoantibodies. Objective: To determine the presence or levels of antibodies (Abs) against matrix metalloproteinase (MMP)-1 and their clinical relevance in LSc. Methods: Anti-MMP-1 Ab was examined by ELISA (Enzyme-Linked ImmunoSorbent Assay) and immunoblotting using human recombinant MMP-1. MMP-1 collagenase activity was determined using biotinylated collagen as substrate and the amount of cleaved biotinylated fragments of collagen by MMP-1 was measured by ELISA. Results: LSc patients exhibited significantly elevated IgG anti-MMP-1 Ab levels relative to normal controls at similar level of patients with systemic sclerosis (SSc). However, IgG anti-MMP-1 Ab levels were comparable among the 3 LSc subgroups: morphea, linear scleroderma, and generalized morphea. When absorbance values higher than the mean + 2S.D. of normal controls were considered positive, IgG or IgM anti-MMP-1 Ab was found in 46% and 49% of total LSc patients and SSc patients, respectively. Anti-MMP-1 Ab was detected most frequently in morphea patients (60%), followed by linear scleroderma patients (47%) and then generalized morphea patients (25%). LSc patients positive for IgG anti-MMP-1 Ab had elevated levels of IgG anti-single-stranded DNA Ab, IgG anti-nucleosome Ab, and shorter disease duration relative to those negative. The presence of anti-MMP-1 Ab in LSc patients was confirmed by immunoblotting. IgG isolated from LSc patients' sera positive for IgG anti-MMP-1 Ab by ELISA inhibited MMP-1 collagenase activity. Conclusion: These results suggest that anti-MMP-1 autoantibody is a novel autoantibody in LSc. c 2008 Japanese Society for Investigative Dermatology., Journal of Dermatological Science, 52(1), pp.47-54; 2008

Localized scleroderma is characterized by idiopathic fibrosis of the skin and adjacent structures, and muscle involvement occurs predominantly in deep morphea. We report a patient with linear scleroderma who presented with slowly progressive atrophy, muscle weakness, and loss of function of her right arm, mimicking a neuromuscular disorder. Muscle biopsy eventually revealed zones of myositis, compatible with morphea profunda. Morphea profunda may thus present as a neuromuscular mimic, even in case of nonprogressive skin sclerosis. Myositis in morphea profunda is generally limited to one region, whereas inflammatory myopathies generally cause diffuse proximal muscle weakness and atrophy. Furthermore, skin changes in morphea profunda differ from those seen in dermatomyositis, and histological features of muscle biopsy can further distinguish between morphea profunda and inflammatory myopathies. Muscle biopsy in morphea profunda implies the risk of sampling error, whereas results of electromyography and muscle imaging might better represent the extent of muscle inflammation.

Localized scleroderma is an inflammatory disorder affecting the skin and underlying tissues, a certain subset of which develops other autoimmune diseases on the basis of a prominent autoimmune background. We here report a unique case of linear scleroderma presenting with a sclerotic plaque on the left thigh, multiple lymphadenopathy in bilateral inguinal and para-aortic lymph nodes, and hepatosplenomegaly, who later developed polymyositis. We describe the detailed disease course of our case and discuss the clinical significance of multiple lymphadenopathy in localized scleroderma based on a review of published work.

We describe a 2-year-old African-American boy with a 4-month history of gradually worsening unilateral edema that was initially noted on his left hand and then approximately 2 weeks later on his left lower extremity. In addition, linear hypopigmented patches were noted along the left forearm and leg, with no appreciable scarring or induration. The edema on the left-hand side of his body progressed so that he developed tense bullae on his left hand. Two months later, the hypopigmented patches were indurated and bound-down, especially over the left groin and thigh. A biopsy specimen from this area showed features characteristic of morphea. In this patient, dilated lymphatic channels secondary to the sclerosis of the morphea caused the bullae. Bullous morphea is a rare condition. We were unable to find any reports its occurrence in children under 18 with associated lymphedema. This entity should be included in the differential for acquired unilateral edema in children.

Serum levels of B-cell activating factor belonging to the tumor necrosis factor family (BAFF), a potent B-cell survival factor, are elevated in patients with systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and systemic sclerosis (SSc). The objective of this study was to determine serum BAFF levels and relate the results to the clinical features in patients with organ-specific autoimmune diseases of the skin, such as localized scleroderma and autoimmune bullous diseases. Serum BAFF levels were examined by enzyme-linked immunosorbent assay in 44 patients with localized scleroderma, 20 with pemphigus vulgaris/pemphigus foliaceus, 20 with bullous pemphigoid and 30 healthy controls. Twenty patients with SSc and 20 with SLE were also examined as disease controls. Serum BAFF levels were elevated in localized scleroderma patients compared with healthy controls. Concerning localized scleroderma subgroups, patients with generalized morphea, the severest form of localized scleroderma, had higher serum BAFF levels than linear scleroderma or morphea patients. The BAFF levels of generalized morphea were comparable with those of SSc or SLE. Furthermore, serum BAFF levels correlated positively with antihistone antibody levels and the severity of skin lesion as well as the number of skin lesions. By contrast, serum BAFF levels were not significantly elevated in patients with pemphigus or pemphigoid. These results suggest that BAFF may be contributing to autoimmunity and disease development in localized scleroderma. © 2007 The Authors Journal compilation © 2007 Blackwell Munksgaard.

Background: Linear scleroderma, also known as “ en coup de sabre,” is a subtype of localized scleroderma that warrants aesthetic correction because it appears on the forehead region in children. Objective: To report dermal fat grafting as a novel and effective surgical treatment option in linear scleroderma. Methods: Under local anesthesia, a dermal fat graft was successfully placed into a subcutaneous pocket that was prepared underneath the depressed scar. The donor site was closed primarily. Results: No early or late complications developed postoperatively. After 1-year follow-up, the dermal fat graft was viable, the depressed scar was adequately augmented, and a good aesthetic result and patient satisfaction were obtained. Conclusion: We believe that dermal fat grafting is a cost-effective option and provides a long-lasting aesthetic outcome in the management of linear scleroderma.

BACKGROUND: To evaluate ophthalmic involvement in a long-term series of patients with en coup de sabre (ECS) close to the eye based on the hypothesis that this is not commonly affected, or simply under-reported. METHODS: An observational study of ophthalmological findings in patients from Copenhagen University Dermatology Clinics. A standard eye examination further included exophthalmometry, axial length and keratometry (IOLMaster), and horizontal eye muscle thickness (B-scan ultrasonography). RESULTS: Thirty-one consecutive patients were included from 2014 to 2015 (25 females, 6 males; median age, 33 years; range, 11–71 years). Twenty-seven patients had undergone ophthalmic evaluation more than once (observation time, 1–31 years; median, 7 years). Most eyes were normal or had currently adapted to eventual adnexal lesions and to insidious changes in eye position and/or motility. However, significant ipsilateral complications had developed related to 8 eyes, where two patients had more than one disorder. The ophthalmic pathologies were: blind eye (n = 2) due to adult age keratopathy/perforation and to Coats-like retinal detachment in childhood; restricted eye motility and diplopia (n = 2); acquired corneal astigmatism (n = 2); and dense cataract with light sense only (n = 1). Two patients had optic neuritis-like presentations, and lacrimal sac pathology occurred in one. CONCLUSIONS: The main ophthalmic focus possibly explained the high proportion of significant lesions in this patient series (in 8 of 31). In addition to the established feature of enophthalmos, the oculometric evidence suggested smaller eye and rectus muscle involvement, interpreted as a secondary (late) negative trophic effect of the overlying skin disorder on eye structures.

Introduction Linear scleroderma morphea is a subtype of localized scleroderma which mainly affects children and is sometimes complicated by bone and joint deformities. Its location at the foot is rarely reported. Aim of study Describe the clinical and anatomical deformity of the foot and ankle in children with linear scleroderma and chat support, from a clinical case and a review of the literature. Observation An 8-year-old boy followed for Linear morphea was addressed to the consultation of physical medicine and rehabilitation for rehabilitation care and casting of a deformation of the right foot. Physical examination found skin lesions in the thigh and right ankle, a paretic right lower limb, an inequality of length of 3 cm lower right limb, with stiffness in his right ankle. Walking is done with lameness and attack the forefoot, the review found podoscopic grade hollow legs [1] with calcaneal valgus more pronounced on the right. The management consisted of a background treatment with corticosteroids, a functional rehabilitation and suitable equipment. Discussion/conclusion Scleroderma focal length of the child's foot is a rare and can be associated with irreducible and scalable orthopedic deformities. Early diagnosis, extensive surgical release in the event of severe and progressive orthopedic deformation, followed by physiotherapy and extended equipment, represent the main elements of the management.

Facial asymmetry is found in patients with or without cosmetic facial alterations. Some patients have facial asymmetry that manifests underlying skeletal problems, while others have only limited soft-tissue facial asymmetry. Orthognathic surgery brings about a dermatic change, as soft tissue covers underlying bones. Limited soft-tissue asymmetry, meanwhile, is difficult to correct. The treatment modalities for the creation or restoration of an esthetically pleasing appearance were autogenous fat grafts, cartilage graft, and silicon injections. A young female patient had right-side facial asymmetry. The clinical assessment involved visual inspection of the face and palpation to differentiate soft tissue and bone. Although the extra-oral examination found facial asymmetry with skin atrophy, the radiographic findings revealed no mandibular atrophy or deviation. She was diagnosed as localized scleroderma with muscle spasm. In conclusion, facial asymmetry patients with skeletal asymmetry can be esthetically satisfied by orthognathic surgery; however, facial atrophy patients with skin or subdermal tissue contraction need treatment by cosmetic dermatological surgery and orthodontic correction.

Linear atrophoderma of Moulin (LAM) is an acquired skin condition that manifests in early childhood and adolescence. It likely represents a form of cutaneous mosaicism that presents with linear, hyperpigmented and atrophic lesions appearing on the trunk and limbs. Its clinical appearance varies and may closely resemble that of atrophoderma of Pasini and Pierini (APP) and linear scleroderma. LAM usually follows a benign course and no effective treatment options exist. We present a case of a young and healthy patient that developed such lesions on her upper and lower extremities over 5 years. The initial clinical impression of linear scleroderma was reviewed in favor of LAM following histological examination of the lesions which revealed no significant inflammatory changes. LAM remains a rare and possibly under recognized entity with reports confined only to the dermatologic literature. This case highlights the importance of recognizing LAM and distinguishing it from linear scleroderma given the significant differences in management and prognosis.

Objectives Juvenile localized scleroderma (JLS) usually has its onset during later childhood. This report describes the clinical and serologic features of six children with congenital localized scleroderma (CLS). Study design A large, multinational study was conducted among pediatric rheumatology and dermatology centers by collecting information on demographics, family history, triggering environmental factors, clinical features, laboratory reports, and treatment of patients with JLS. Patients with onset at birth were carefully examined. Results Among 750 patients with JLS, 6 patients (0.8%) had scleroderma-related lesions at birth. Female-to-male ratio was 2:1. All patients had linear scleroderma, in four involving the face with en coup de sabre appearance. Two patients were misdiagnosed as having skin infection, one nevus, one salmon patch, and two undefined skin lesions. The mean diagnostic delay was 3.9 years. In comparison with the group of 733 patients with late-onset JLS, CLS presented a significantly more prolonged disease duration at diagnosis and a higher frequency of en coup de sabre subtypes. Conclusions Congenital localized scleroderma is a rare and probably underestimated condition in neonates. The linear subtype was the exclusive manifestation of the disease. CLS should be included in the differential diagnosis of infants with cutaneous erythematous fibrotic lesions to avoid functional and aesthetic sequelae and to allow prompt therapy.

Background Progressive facial hemiatrophy (PFH) is characterized by a slowly progressive atrophy of soft tissues and in some cases bony structures. Coexisting features of localized scleroderma (LS) are commonly observed, indicating the close nature of both disorders. Objective We sought to investigate clinical, serological, and radiographic findings in PFH from 278 patients with LS and to discuss the relationship to linear scleroderma en coup de sabre (LSCS). Method A total of 12 patients with PFH were retrospectively evaluated on the basis of clinical, serological, and radiographic findings. Results Five patients (42%) presented with complete PFH, and 7 patients (58%) with partial PFH involving either cheek or forehead. Five of the patients (42%) had a coexisting LSCS lesion, and 3 of them (25%) had concomitant LS of the trunk. Intraoral involvement was present in 6 cases. Neurological involvement was common, in particular, epileptic seizures. Serologic investigations showed neither evidence for infection with Borrelia burgdorferi nor any other indication of underlying systemic immunological disorders. Limitations There were a relatively small number of patients in a retrospective study. Conclusion The reported cases suggest a close relationship between PFH and LSCS. PFH might appear as two different subtypes, one involving cutaneous structures and presenting with clinical features similar to LSCS, one being strictly restricted to subcutaneous structures, primarily affecting the cheek area. The manifold clinical features of central nervous system involvement indicate the pathogenetic importance of neurological involvement in the development of PFH. Magnetic resonance imaging should be included in the tools of standard diagnostic procedures in patients with PFH. The etiologic relevance of autoimmunity as well as preceding trauma should be investigated in larger collective studies.

Linear scleroderma is a subtype of localized scleroderma generally observed in children, and may produce secondary bone and joint deformities. Its localization at the foot or ankle is rarely reported. A complete review of the literature reveals 21 cases of foot or ankle deformity due to linear scleroderma to which we add one case. The clinical and radiological aspects of the deformity, its pathogenesis, histological findings as well as orthopaedic management are presented and discussed.

Background Anti-agalactosyl immunoglobulin G (IgG) antibodies (anti-AG IgG) have been reported to be detected and correlated with disease activity in some collagen diseases. Method Forty-seven serum samples from patients with localized scleroderma were examined using an enzyme-linked immunosorbent assay. Results Anti-AG IgG were positive in 19% of patients with localized scleroderma. The frequency of anti-AG IgG in generalized morphea was much higher than that in linear scleroderma or that in morphea. There was a significant correlation between anti-AG IgG levels and the number of the sclerotic lesions and between anti-AG IgG levels and the number of involved areas. The levels of anti-AG IgG were significantly higher in patients with antinuclear antibody, antisingle-stranded DNA antibody or rheumatoid factor than in those without. Conclusion Anti-AG IgG can be an indicator of the severity of localized scleroderma.

Progressive hemifacial atrophy in Parry-Romberg syndrome is associated with a wide variety of ophthalmologic findings. Progressive visual loss, especially in the first decade, is rare in this condition. We present a young girl originally felt to have linear scleroderma and anisometropic amblyopia, who was eventually diagnosed with Parry-Romberg syndrome as the cause of her cutaneous and ophthalmologic findings.

A 28-year-old woman with alopecia since childhood presented with 4 years of progressive intermittent diplopia and right eye getting “stuck” with upward gaze. Maddox rod test revealed a subtle intermittent right hypotropia and right enophthalmos (figure 1). Asymptomatic T2 hyperintensities were noted on MRI (figure 2). Scalp morphea was confirmed pathologically. Formal rheumatologic evaluation was otherwise unremarkable. Linear scleroderma en coup de sabre presenting as positional diplopia, unilateral enophthalmos, and focal neuroimaging findings is rare and is suspected to represent a chronic local inflammatory process.1,2 The brain lesions tend to develop adjacent to the skin atrophy.1

Scleroderma en coup de sabre, a variant of localized scleroderma, is a disorder of unknown origin characterized by fibrosis of connective tissue. Rare complications of scleroderma en coup de sabre are orbital and intracerebral involvement. We describe a patient with scleroderma en coup de sabre in whom intracerebral and orbital lesions developed after 2 decades of disease duration. Clinically, she had epilepsy, impaired vision, and retro-ocular pain of the affected eye. A 12-month course of interferon-γ stopped progression of visual symptoms caused by orbital fibrous tissue. To our knowledge, this is the first patient with scleroderma en coup de sabre complicated by orbital involvement who was successfully treated with interferon-γ.

A 24 year old woman in the 33rd week of pregnancy developed progressive neurological complications with right sided hemiparesis in association with the occurrence of linear scleroderma "en coup de sabre" (LSCS) and pre-existing plaque-morphea, already being treated by balneophototherapy. Further progression of neurological symptoms led to a caesarean section with the delivery of a healthy child. Brain magnetic resonance imaging (MRI) showed focal T2 signal increases in the left frontoparietal region directly adjacent to the area of LSCS. Cerebrospinal fluid analysis revealed oligoclonal bands, suggesting an intracerebral inflammatory process. Subsequent pulsed corticosteroid treatment led to a remission of neurological symptoms and to a marked resolution of the MRI lesions.

Melorheostosis is a rare disorder characterized by irregular, flowing hyperostosis in long bones, commonly described on radiographs as wax flowing down a candle. In addition to bony sclerosis, cutaneous manifestations overlying the involved bones have been reported including linear scleroderma, neurofibromatosis, and vascular and lymphatic malformations. Unilateral nevoid telangiectasia (UNT) is a rare primarily cutaneous condition characterized by linearly arranged small dilated blood vessels in dermatomal or Blaschkoid patterns on the skin. Here, we present the case of a nine-year-old Korean male with UNT associated with ipsilateral melorheostosis.

Introduction ‘En coup the sabre’, a linear form of facial scleroderma, is rarely complicated by neurological symptoms. The aetiology of the underlying cerebral lesions is still obscure, but there is evidence in favour of a vasculitic process. Our case, with neurological symptoms presenting over ten years, illustrates this picture. Case report A 60 year old woman with history of facial linear scleroderma and migraine, presented to casualty with acute left sided weakness. Two months prior to this, she had been admitted for new onset of seizures. Seven years earlier she had developed acute left hemianopia and a brain scan had revealed a possible arterio-venous malformation (AVM) and gliotic changes in the right hemisphere. A new MRI showed progression of the right sided lesions. A cerebral angiogram did not confirm AVM, but showed signs of vasculitis. Identical oligoclonal bands were found in cerebrospinal fluid (CSF) and blood. ANA and ANCA were positive. She was treated with steroids and started on mycophenolate. She improved and remains stable at 2 years. Conclusions The association between linear scleroderma and neurological symptoms is well known, but its pathological nature is still debated. Vasculitis is a possible underlying process, and needs consideration and treatment in case of symptoms progression.

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 Scleroderma encompasses a spectrum of disorders characterized by thickening of the skin and subcutaneous tissue with increased collagen deposition. Linear scleroderma is subdivided into progressive hemifacial atrophy and en coup de sabre subtype. Objective We report a case of congenital linear scleroderma identified in an adult, misdiagnosed since birth as birth trauma. Methods We completed a review of the literature for similar cases using PubMed and Medline. Results This is the first report of congenital linear scleroderma en coup de sabre diagnosed in an adult following an initial misdiagnosis of birth trauma. The sequelae of linear scleroderma can be significant as it can result in growth retardation, muscle atrophy, contractures, limb length discrepancy, and disfigurement. Conclusions This report emphasizes the importance of educating practitioners about linear scleroderma. Early recognition is key as a delay in diagnosis can result in potentially preventable, irreversible growth defects and disfigurements.

Linear scleroderma en coup the sabre (LSCS), progressive facial hemiatrophy (PFH) and autoimmune encephalitis are distinct clinical entities, although patients with overlapping features have been reported. We performed a multicenter retrospective review of a series of children with LSCS and/or PFH to explore the relation between these entities. The files of 16 children were reviewed, 11 presented with LSCS, 5 with PFH, with time overlapping cutaneous features were seen. Extracutaneous signs were found in both groups. ANA were present in more than 50 % of patients. Almost half of our patients presented with CNS manifestations comprising unilateral headache, migraine and epilepsy with or without abnormalities on MRI. Brain biopsy in one patient was consistent with Rasmussen encephalitis. In two other children, associated autoimmune manifestations were present.Our patient cohort brings more arguments to consider LSCS and PFH as a single disease entity with LSCS and superficial skin involvement at one end of the spectrum and PFH with involvement of subcutaneous deep tissue at the other end. In both entities, encephalitis can be observed. Our findings of circulating ANA, intradermal lymphocytes and IgG, intrathecal IgG production and clinical improvement with immunosuppressive therapy endorse the concept of a possible common immune-inflammatory pathogenesis.• LSCS, PFH and immune-inflammatory encephalitis are distinct clinical entities, but patients with overlapping features have been reported.• We present a unique paediatric cohort with LSCS, PFH and/or encephalitis. • We endorse the concept of a common immune-inflammatory disease process.