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Copyright of Plastic Surgery is the property of Sage Publications Inc. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

ABSTRACT This is the first report of pediatric linear scleroderma successfully treated with the topical Janus kinase (JAK) inhibitor delgocitinib. JAK inhibitors targeting the JAK/STAT pathway have been used to treat various immune‐mediated diseases. In both in vitro and in vivo, JAK inhibitors also block the transforming growth factor (TGF)‐β‐mediated effects that contribute to skin sclerosis. In the present case, the histological findings of inflammation and fibrosis were considered as conditions that would benefit from the anti‐inflammatory and antifibrotic effects of the JAK inhibitor, delgocitinib. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Parry–Romberg syndrome is characterized by progressive dystrophy in one half of the face, which usually begins in childhood. Correct and timely diagnosis of this disease, as well as a multidisciplinary approach and timely surgical treatment to minimize the psychological effects and improve the patient's appearance are of particular importance. Parry–Romberg syndrome is characterized by progressive dystrophy or loss of subcutaneous tissue in one half of the face, which usually begins in childhood and continues with skin changes, and can also be associated with linear scleroderma. Although this disease has been known for more than 150 years, its exact cause and pathogenesis are not well understood. The clinical feature of Parry–Romberg syndrome that makes it possible to diagnose is unilateral idiopathic facial atrophy. The reported case is a 14‐year‐old boy who suffered from hemifacial atrophy of the frontal area since he was 7 years old was referred to a plastic and cosmetic surgery specialist and underwent surgery without systemic symptoms and in the inactive phase of the disease. Correct and timely diagnosis of this disease, as well as a multidisciplinary approach and timely and appropriate surgical treatment to minimize the psychological effects and improve the patient's appearance are of particular importance. [ABSTRACT FROM AUTHOR]

Copyright of Paediatrics & Family Medicine / Pediatria i Medycyna Rodzinna is the property of Medical Communications Sp. z o.o. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract 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

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.

Juvenile localized scleroderma is characterised by inflammation which drives fibrosis in skin and soft tissues. The more severe subtypes of localized scleroderma such as linear and craniofacial are more common in children. Additionally, extracutaneous involvement is seen in half of all children and is associated with poorer treatment outcomes and health-related quality of life. Evidence for the management of craniofacial and extracutaneous involvement is lacking and therefore poses a challenge to clinicians. This review aims to provide a practical approach to management of these most challenging features of juvenile localized scleroderma through case studies where we present the available evidence, current recommendations and considerations for management., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier Ltd.)

Objective The aim of this study was to evaluate factors associated with extracutaneous involvement (ECI) in juvenile localized scleroderma (jLS). Methods A prospective, multicentre, 6-month observational study was performed. The data collected included disease features, global assessments, and subject symptoms. Bivariate and linear multilevel regression analyses were performed. Results A total of 86 jLS subjects (80% female, 80% Caucasian), median age of disease onset 7.7 years, were evaluated. Most had linear scleroderma or mixed morphea. Of the 86 subjects, 49 (57%) had 125 extracutaneous problems {median 2 [interquartile range (IQR) 1, 3] per subject} from nine organ systems. Most of these subjects had multiple musculoskeletal problems. ECI was associated with more extensive cutaneous involvement, higher number of symptoms, family history of autoimmunity, and ANA and RF positivity. Subjects with ECI had higher scores for physician global assessment of damage (PGA-D), and parental global assessment of disease impact, but not baseline physician global assessment of disease activity (PGA-A). Although subjects with ECI received more MTX and glucocorticoid treatment, they had a slower reduction in PGA-A scores and symptoms over time, suggesting a poorer response to treatment. In logistic regression modelling, female sex had the largest effect on parental impact scores. Conclusion ECI occurred in the majority of subjects with jLS, and was associated with more medication use, longer treatment duration, higher PGA-D scores, and higher parental assessment of disease impact. Our findings suggest that jLS subjects with ECI have greater overall disease burden, both cutaneous and extracutaneous, and poorer response to treatment. More study of the treatment needs of this population is warranted. [ABSTRACT FROM AUTHOR]

Abstract 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.

Abstract 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: 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 < 0.05). 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. [ABSTRACT FROM AUTHOR]

Copyright of Dermatology Review / Przeglad Dermatologiczny is the property of Termedia Publishing House and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

• Parry Romberg syndrome is a neurocutaneous disease characterized by facial hemiatrophy and variable neurological symptoms like seizure disorder and headache. • Ipsilateral cerebral hemi atrophy, abnormal white matter T2 hyperintensities and intra-parenchymal calcification are the most frequent neuroimaging abnormality. • Parry Romberg Syndrome and linear scleroderma represents the spectrum of same disease and differentiation maybe difficult. Parry Romberg syndrome (PRS) is a distinct sporadic self-limiting entity comprising progressive hemi- atrophy of the face with predisposition to affect the skin, subcutaneous tissue and in some cases deeper extension to the cartilage, osseous structures and musculature (Wong et al., 2015; Aydın et al., 2015; Sharma et al., 2012) [1–3]. Neurologic and ocular involvement is variable as is the progression of this entity. With a multitude of aetiologies ranging from autoimmune to infectious this syndrome is a diagnosis of exclusion, with a highly variable prognosis, drawing the amusement of radiologists and clinicians. Radiological assessment and subsequent diagnosis of this entity can aid in alleviating the lifestyle hampering progression of this disease process. We hereby report 4 cases of this syndrome and aim to describe the varied radiological presentations of this entity with concise review of literature. [ABSTRACT FROM AUTHOR]

Objective: Localized scleroderma (LS), including morphea and linear scleroderma, is an autoimmune disease where excessive subcutaneous collagen deposits lead to thickening, scarring, and fibrosis of the tissues. LS coexisting with inflammatory arthritis is less well-described but has been reported in as many as 20% of 53 LS patients in a recent cohort. Herein, we describe a cohort of 8 children with both LS and inflammatory arthritis. The objective of this study is to determine the characteristics of inflammatory arthritis in children with LS and their response to treatment regimens. Methods: A retrospective chart review was completed on patients less than 19 years of age who were diagnosed with either morphea or linear scleroderma at the Children of Alabama center from 2004-2018. Patients were identified using ICD-9 and ICD-10 diagnostic codes. Records were reviewed for additional diagnostic codes, exams, and laboratory findings confirming coexisting inflammatory arthritis and LS. Results: A total of 87 patients with a diagnosis of either morphea or linear scleroderma were identified. Eight (9%) had coexisting inflammatory arthritis according to the diagnostic codes with documented active arthritis. Median age of initial rheumatic disease diagnosis was 7.5 years. A majority of patients with both LS and inflammatory arthritis were female (62.5%). Half of the patients (n=4, 50%) had LS lesions over arthritic joints. All of the identified patients were diagnosed with a form of juvenile idiopathic arthritis (JIA). The JIA diagnoses varied widely in 3 (37.5%) patients with rheumatoid factor (RF) negative polyarticular JIA, 2 (25%) with oligoarticular JIA, 2 (25%) with psoriatic JIA, and 1 (12.5%) with enthesitis-related JIA. The timing of onset of LS and inflammatory arthritis varied widely. Three (37.5%) patients had LS lesions preceding clinical arthritis, and three (37.5%) had arthritis before the appearance of LS. Two (25%) patients had both LS and arthritis at the time of diagnosis. All patients received methotrexate (MTX) during their disease course with only 3 (37.5%) receiving systemic steroids during treatment. All 8 patients showed resolution of LS lesions. However, 6 of the 8 patients demonstrated active arthritis on combination MTX and TNFi therapy. Conclusion: In this cohort of pediatric LS, 9% of patients had coexisting inflammatory arthritis. The characteristics of this cohort varied widely. All patients received MTX initially and showed a resolution of LS lesions. However, in the majority of patients, the arthritis failed to respond to MTX and TNFi combination therapy. These results suggest that inflammatory arthritis coexisting with LS may be less likely to respond to traditional inflammatory arthritis or JIA therapies. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Localized scleroderma is a connective tissue disorder that causes excessive collagen deposition and skin fibrosis. It can be subdivided into morphea and linear scleroderma. En coup de sabre (ECDS) is a rare variant of linear scleroderma typically found among children. It is usually treated with methotrexate and corticotherapy in addition to folic acid supplements. To date, few cases of ECDS have been reported with oral involvement. This case report discusses a seven-year-old girl with linear scleroderma ECDS who was referred to the dental clinic to evaluate muscular hypotrophy on the floor of the mouth. Upon clinical and radiographic examination, the patient had hypotrophied mylohyoid muscle, reduced alveolar bone height on the affected side, and a deviated midline to the affected side as well. Furthermore, the patient was classified as having a high caries risk. After consultation with the primary physician regarding treatment modalities and options, the patient completed her comprehensive dental treatment at the Dental Department at King Faisal Specialist Hospital and Research Centre in Riyadh, Saudi Arabia., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Alkanhal et al.)

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Localized facial scleroderma usually presents as frontal linear morphea or progressive hemifacial atrophy. Only isolated cases of trigeminal painful neuropathy have been described.Case Report: A 43-year-old woman developed an oval lesion on the right cheek. After 1 year, she noticed constant "pulling" pain and episodes of lancinating pain, both spontaneous and triggered by chewing and cold drinks. She was diagnosed with solitary morphea profunda and CT scan, ultrasonography, cranial MRI and biopsy were completed. Methylprednisolone (1 gr/day for 3 days) was prescribed. For pain, gabapentin, oxcarbazepine, amitryptiline, pregabalin and eslicarbacepine were all ineffective. A capsaicin patch was placed with prolonged benefit. Later on, the pain slightly worsened; occipital blockade was effective and methotrexate was recommended.Conclusion: This is the first case of solitary morphea profunda associated with painful trigeminal neuropathy. Treatment should include immunosuppressants and treatment of neuropathic pain, in which local therapies seem particularly beneficial. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Linear scleroderma has been associated with muscle spasms ipsilateral to skin lesions. Typically, spasms are generated by trigeminal innervated muscles, leading to hemi-masticatory spasm (HMS). Case Report: We report a case of linear scleroderma associated with spasm of muscles innervated not only by the trigeminal, but also by the facial nerve. Discussion: We review the patient’s successful treatment with incobotulinumtoxinA, a formulation of botulinum toxin that has not been reported for use in this condition.

Linear scleroderma (LS) is clinically characterized by the presence of sclerotic areas of skin, which develop in a linear pattern. Primary sclerosing cholangitis is a cholestatic disorder that can lead to end-stage liver disease. We present, for the first time in English literature, the case of a patient suffering from both the diseases. This highlights the fact that, even though LS has conventionally been considered to be a form of localized scleroderma, this does not necessarily imply that it is an exclusively cutaneous disease.

Abstract is missing (Short communication)

Introduction: Immune-mediated necrotizing myopathies (IMNMs) are characterized by progressive weakness, elevated serum creatine kinase levels, and necrotizing myopathic features on muscle biopsy. Presence of highly specific autoantibodies against signal recognition particle (SRP) or 3-hydroxy-3-methylglutaryl- coenzyme A reductase (HMGCR) can aid in recognition and confirmation of IMNMs.Methods: In this study we describe a boy with HMGCR-positive necrotizing myopathy and highlight the clinical features of the patient.Results: In contrast to most adults, the patient described had a more indolent disease course, reminiscent of a muscular dystrophy. Intravenous immunoglobulin monotherapy resulted in a dramatic clinical response with return to normal strength.Conclusions: Systematic consideration of IMNMs and testing for relevant autoantibodies in children with suspected but genetically unconfirmed muscular dystrophy may help improve diagnostic accuracy and allow timely treatment with potentially highly effective immunotherapies. Muscle Nerve 56: 175-179, 2017. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]