Hemiatrophy Research Articles

Localized scleroderma

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

Neuropathologic findings in a patient with Parry-Romberg syndrome: active cerebral vasculitis and brain injury

PurposeParry-Romberg syndrome (PRS) is a rare disorder characterized by slowly progressive hemifacial atrophy. Although the underlying etiology is unknown, proposed disease mechanisms which include autoimmune, infection, trauma, and other causes have been proposed as underlying disease mechanisms. Approximately, 10–20% of PRS patients have neurologic manifestations such as epilepsy, headaches, or associated vascular malformations. There are reports of PRS responsive to immunosuppressive medications, supporting the autoimmune hypothesis. Currently, the neuropathologic findings in patients with PRS are not well documented.MethodsHerein, we present a case of a 19-year-old female with PRS, who underwent partial frontal lobectomy for intractable epilepsy.ResultsThe resection specimen showed multifocal active lymphocytic (T-cell mediated) arteritis in midsized cortical arterioles, with the adjacent meninges showing fibrosis involving both the meningeal tissue and its vascular network. In addition, neuronal loss and gliosis were evident in the cortex, likely due to the associated vascular injury.ConclusionsThis report is the first to demonstrate active cerebral vasculitis in a patient with PRS, supporting the previous suspicion of inflammatory etiology in this disease. In addition, the widespread vascular fibrosis in the meningeal vessels and an area of cortical ischemia support the presence of previous inflammatory vascular processes in the area.

Airway Ultrasound Findings in a Rare Case of Parry–Romberg Syndrome

Parry–Romberg syndrome (PRS) is a rare disorder characterised by progressive hemifacial atrophy, along with various neurological and ophthalmological manifestations. Patients with PRS may undergo reconstructive procedures such as fat grafting to address the facial contour deformities. Anaesthesiologists may face significant challenges due to the asymmetric craniofacial structures and the potential presence of neurological deficits, including hemiparesis. We report a case of an 18-year-old male with left-sided hemifacial atrophy and PRS with wasting of one side of the lobe of the thyroid gland who underwent fat grafting under general anaesthesia. The challenges faced during the anaesthetic management of this patient are airway management, positioning and monitoring of neurological deficits. PRS has been reported to coexist with other autoimmune diseases, including thyroid disorders. However, thyroid involvement in PRS is rare. This case used ultrasound to evaluate the airway and assess PRS impact. This case highlights the importance of ultrasound airway, which can serve as an effective tool for identifying associated conditions (like thyroid atrophy) in patients with PRS and can assist in the diagnosis and management of this rare and complex disease.

Application of free serratus anterior muscle-fascial composite tissue flap and facial lipofilling in repairing progressive hemifacial atrophy

IntroductionProgressive hemifacial atrophy (PHA) is a nonnegligible disease, and its treatment currently lacks consensus. We aim to conduct an analysis of PHA patients to summarize the postoperative effect. Moreover, we introduced the free serratus anterior muscle-fascial composite tissue flap as a safe and novelty surgical procedure for moderate-severe PHA. MethodsThis clinical study included four patients who received a free serratus anterior muscle-fascial composite tissue flap and 19 patients who received Coleman fat transplantation. Preoperative (preoperative photograph and imageological examination) and postoperative (postoperative photograph, complications, therapeutic effect, and satisfaction) assessments were performed for all PHA patients. Body Image Concern Inventory (BICI), Self-rating Anxiety Scale (SAS), Self-rating Depression Scale (SDS) were performed preoperatively and postoperatively. ResultsAll the cases were cured with a good appearance with two kinds of operations. Free serratus anterior muscle-fascial composite tissue flap could correct face defects in one surgery and achieve good long time and short-time postoperative satisfaction in moderate-severe PHA. Fat transplantation could also enhance appearance in numerous operations for mild-moderate PHA. The volume of free-fat grafts decreased obviously after implantation in many cases. So, many patients (42.11%) accepted a series of operations to achieve satisfied postoperative effect. BICI, SAS, SDS score decreased a year later in all patients. ConclusionFree serratus anterior muscle-fascial composite tissue flap transplantation is an effective and safe treatment for moderate to severe PHA.

Parry-Romberg Syndrome: Successful Result in a Single Procedure Combining Silicone Implant and Structural Fat Grafting for Severe Facial Deformity.

Parry-Romberg syndrome (PRS) is an enigmatic and acquired degenerative condition. It is characterized by a gradual and progressive facial hemiatrophy, after which facial deformities appear unexpectedly in a stable state. The degree of disfigurement depends on tissue involvement, illness onset, and duration. Nowadays, non-invasive treatments with lower morbidity, such as structured fat grafting (SFG) are preferred over traditional operations such as free flaps and bone grafts. However, for severe cases of PRS, multiple sessions of SFG are required. Despite the therapeutic failure that a 20-year-old lady had suffered after five previous conventional surgical procedures, the outcome was successful after only one surgical therapy employing a particular combination of SFG and malar silicone elastomers implant (SI). The author highlights the interest in a combination of non-invasive procedures using SFG and malar augmentation with SI to repair large volume loss while improving symmetry and skin texture. Otherwise, the role of adipose-derived stem cells in fat survival is crucial and should be the focus of future studies and the key to technological advancement.

Transcriptome Sequencing of the Oral Mucosa Reveals Gene Expression Differences in Patients With Parry-Romberg Syndrome

Objective: Parry-Romberg syndrome (PRS) is an acquired disease characterized by progressive unilateral atrophy of the facial skin, subcutaneous tissue, muscle, and bone. There are various hypotheses to try to explain the occurrence of the disease, but the specific etiology and pathogenesis remain unclear. This study aimed to explore the potential molecular pathogenesis of the disease by using next-generation RNA-sequencing technology. Methods: The authors collected oral mucosal tissue from the affected side and the healthy side from 3 patients with PRS. Tissue samples were subjected to RNA extraction, whole transcriptome sequencing, and bioinformatics analysis. Differentially expressed genes were obtained from both groups of samples and then analyzed for functional enrichment. Results: A total of 186 differentially expressed genes were screened from the 2 groups of samples. Compared with the healthy side, several immune-related genes, including immunoglobulin kappa variable (IGKV)2D-28, IGKV1D-33, IGKV1-33, and NLRP10, were significantly upregulated in the affected tissue. In addition, the differential genes were significantly enriched in metabolic pathways including pancreatic secretion, protein and fat digestion, and absorption. Conclusions: The authors described the gene expression differences between the affected and healthy tissues of patients with PRS for the first time. Immune responses may play a role in the pathogenesis of PRS.

A Novel Method for one-Stage Repair of the lip Vermilion Defects in Progressive Hemifacial Atrophy

Background Progressive hemifacial atrophy often causes lip vermilion defects in patients. In this study, we described a one-stage repair method for lip defects in progressive hemifacial atrophy using a lip vermilion mucosal flap or combined dermal fat flap graft. Patients and Methods Patients diagnosed with progressive hemifacial atrophy with lip vermilion defects from 2010 to 2022 were included in this study. Based on the severity and location of the patient's lip defect, a lip vermilion mucosal flap was designed and transferred to the lip defect or combined with a hip dermal fat flap for one-stage repair of the lip morphology. Lip morphology and function of patients were followed up after surgery. Results A total of 22 patients were enrolled in this study, including 15 patients with lip defects on the upper lip alone and 7 patients with both upper and lower lip defects. Follow-up six months to two years postoperatively, all patients recovered uneventfully without complications. The repaired lips of the patient had a full and symmetrical morphology with no visible scarring. Two patients experienced transient dysesthesia of the lips postoperatively and both returned to normal after three months. All patients had good lip closure with normal dietary and speech function. Conclusions The method we described for repairing lip defects in progressive hemifacial atrophy can achieve satisfactory aesthetic and functional lip results. The distinct advantage of this approach is that the patients undergo only one-stage operation and it can be used to repair both upper and lower lip defects.

A Surgical Management Algorithm for Type 4 Parry-Romberg Syndrome.

Type 4 Parry-Romberg syndrome (PRS) affects multiple facial esthetic units involving many tissue types, requiring both bone and soft tissue restoration and requiring different surgical procedures over a long period of time. To date, the timing and sequence of these operations remains controversial. A retrospective analysis was performed to introduce our surgical management algorithm for type 4 PRS and to evaluate its safety and efficacy according to long-term follow-up results. Six Chinese patients (5 females and 1 male) fulfilled the inclusion criteria. The follow-up period ranged from 23 to 90 months (mean 51.83mo). All patients were satisfied with the improvement in facial appearance after staged bone and soft tissue reconstruction. No major complications were reported except for 1 case of infection after Medpor implantation. For severe PRS, both bone and soft tissue reconstruction is recommended. Enlargement of the bony framework provides a good foundation for subsequent soft tissue restoration. Alloplastic implants in the maxilla can be problematic if the patient has undergone either a zygomatic or maxillary osteotomy. The anterolateral thigh adipofascial flap is a reliable choice for large soft tissue deficits in type 4 PRS. We hope that reporting our surgical management algorithm with long-term follow-up results will improve the personalized treatment of these patients.

P08 Appearance matters: a case of adult-onset Parry-Romberg syndrome with linear scleroderma en coup de sabre and neurological involvement

Abstract Introduction We describe the case of an adult patient who initially presented with neurological symptoms. Investigations were suggestive of a neuroinflammatory disorder but the diagnosis was unclear. It was only as time passed that the cutaneous signs and symptoms of Parry-Romberg syndrome (PRS) and linear scleroderma en coup de sabre (LSCS) became apparent to clinicians as the underlying cause for her presentation. Case description This female patient, with no past medical history, presented in 2015 when she was 43 years old. She reported a one-day history of malaise and lethargy, left-sided headache, numbness affecting the right face and right arm and dysphasia. Her symptoms gradually improved over the following few days. An MRI brain showed extensive T2 hyperintensity in the right frontal and frontoparietal regions. Cerebrospinal fluid (CSF) showed a lymphocytosis and oligoclonal bands, which were present in the CSF only. A repeat MRI brain a few weeks later showed slightly less extensive changes. This was treated by neurologists as a neuroinflammatory disorder with intravenous methylprednisolone pulses. At follow-up, she mentioned that she had noticed a change in her appearance, with the right side of her face becoming increasingly asymmetrical over the last few years. Neurologists were puzzled regarding the diagnosis, but given that she was almost asymptomatic at review, they decided to monitor her with serial MRI scans. There were no further neurological episodes and no interval changes on repeat scans. In 2020, she was referred to ophthalmology, as she had noticed that her right eye had become increasingly sunken. MRI of the orbits and sinuses was reported to show enophthalmos of no obvious cause. Around the same time, she was also referred to dermatology for an enlarging patch of alopecia over the right frontoparietal scalp. Autoimmune screening blood tests including ANA, ENA, dsDNA and centromere were negative. Scalp biopsy was inconclusive but was most in keeping with old alopecia areata. She was treated with intralesional steroids and had a partial response. It was only by 2021 that PRS with LSCS was recognised as the cause of her symptoms. We can be confident that her neuroinflammation is an extracutaneous manifestation, which occurred early on when cutaneous signs were subtle. Discussion PRS is a rare, acquired disorder characterised by progressive unilateral atrophy of facial skin, soft tissue, muscles, and underlying bony structures. LSCS is a subtype of linear scleroderma that occurs on the forehead or frontoparietal scalp. PRS and LSCS commonly coexist, and it is debated whether they are on a spectrum of the same disease. Both usually occur in childhood or adolescence, and adult-onset disease is very rare. The diagnoses are clinical and there are no specific diagnostic tests. Both are associated with neurological involvement, including seizures, headaches, focal neurological deficits and neuropsychiatric changes. Usually, the cutaneous lesion develops months to years before the onset of neurological symptoms. Our patient had noticed subtle changes in her appearance at least five years before her presentation in 2015. Neuroimaging abnormalities are predominately ipsilateral to the cutaneous lesions but may rarely be contralateral. Key learning points Given that adult-onset PRS and LSCS are very rare, there may be a lack of awareness of the conditions amongst adult physicians. This can lead to diagnostic delay. This case highlights that PRS and LSCS are not just conditions of the skin and connective tissues and that neurological involvement is a frequent extracutaneous manifestation. This case is also a reminder of the importance of a thorough examination of the skin, especially when the diagnosis is unclear.

Parry Romberg Syndrome: A Rare Entity from a Primary Care Center in Bangladesh

Parry Romberg syndrome (PRS) is an acquired, rare, neurocutaneous disorder presenting most commonly as hemiatrophy of face with characteristic involvement of skin, subcutis, bone and denture with or without neurological manifestations.Here we present a case of 16 year old girl, who presented with characteristic insidious, progressive, selflimited hemifacial atrophy accompanied with headache and facial pain. Bangladesh Med J. 2021 Sept; 51(1): 59-62

En coup de sabre linear scleroderma – diagnostic difficulties. Case report

Frontal linear scleroderma (morphea en coup de sabre) is a rare disease from the group of limited connective tissue diseases, most often affecting children. Due to the fact that it is not a common dermatosis, diagnosis and treatment may be difficult. Therefore, we believe it is advisable to present the diagnostic difficulties in a patient of the Provincial Integrated Hospital in Elbląg. It is also noteworthy that the patient’s neurological symptoms preceded the appearance of skin lesions. Clinical symptoms and imaging studies led us to observe progressive hemifacial atrophy. The patient requires interdisciplinary specialist care and diagnosis in the field of neurology, dermatology, rheumatology and ophthalmology.

Progressive Facial Haemiatrophy (Parry–Romberg Syndrome) Treated with Hyaluronic Acid Fillers

Introduction: Parry–Romberg syndrome (PRS) is a rare neurocutaneous condition that can affect the skin, subcutaneous fat, muscle, and bone on one side of the face. PRS presents with progressive but self-limiting facial haemiatrophy, and its severity ranges from barely noticeable asymmetry to severe disfigurement. The authors describe a 35-year-old male with right facial haemiatrophy. The patient presented in a stable condition at the initial consultation. Objectives: Adipose tissue autografts were the most used tool to treat many congenital or acquired facial deformities. The authors proposed using cross-linked hyaluronic acid (HA) fillers over those traditionally used to correct facial deformities due to PRS. Discussion: PRS is usually self-limiting, with the maximal progression of the disease 2–5 years after onset. There is no specific cure for PRS at present. After 7 years of progression from the onset, this patient entered a stable phase. Reconstructive treatment was then indicated, since there were no further signs of atrophy. Since adipose tissue autografts require complicated surgical skills, which take a lot of time and cost, the authors report a case of PRS augmented by HA filler in a 35-year-old male patient to suggest that HA filler could be a safe and simple alternative to surgical treatment. The authors conclude that HA fillers are a safe tool for treating facial deformities in patients with PRS.

Progressive hemifacial atrophy or Parry Romberg syndrome case report and literature review

Parry-Romberg syndrome is a condition characterized mainly by left-sided hemifacial atrophy that affects skin, subcutaneous tissue, muscle, and bone. The pathophysiology is not established and several theories have been proposed, among which lymphocytic neurovasculitis manifested as a chronic vascular lesion measured by incomplete endothelial regeneration stands out. The actual treatment consists of autologous fat transfer, making voluminous grafts to restore symmetry and improve the quality of the skin. An overcorrection is recommended to compensate for the reabsorption estimated of up to 80%. The treatment consists of repeated applications until correction is achieved, there is currently no consensus regarding the preparation of the graft. The objective of this work is to propose an alternative to conventional treatment by enriching fatty tissue with platelet-rich plasma, which favors the formation of extracellular matrix, collagen fibers, and angiogenesis in an accelerated manner. We conclude that the use of platelet-rich plasma significantly improves skin quality and fat graft integration.

Parry–Romberg syndrome—Still progressing over 20 years of disease course

AbstractParry–Romberg syndrome presents with atrophy of one‐half of the face involving skin, soft tissue, muscle, and bone. It usually progresses slowly over 2–20 years and finally stabilizes. Neuropsychiatric abnormalities are subtle, seen in about 10% of the patients, and include cognitive disturbances, aphasia, hallucination, and other psychiatric disorders. We describe the case of a young female who had progressive brain atrophy over 20 years of disease course and presented with focal seizures and recent onset progressive cognitive impairment.

Mysterious Association of Systemic Lupus Erythematosis with Perry Romberg Syndrome

Parry Romberg syndrome (PRS), also referred to as progressive hemifacial atrophy, progressive facial hemiatrophy, or idiopathic hemifacial atrophy, was first described by C Parry and M Romberg.1 It manifests in the first two decades in morphologically normal-born individuals. It commonly affects one or more dermatomes in the trigeminal nerve territory. It has an early onset of ophthalmic and neurological involvement and a variable maxillo-facial or cardiac involvement. It is an idiopathic, gradually progressive craniofacial asymmetry, following the atrophy of subcutaneous tissue, muscles, osseous, and cartilaginous structures. Ophthalmic involvement occurs in up to 35% of cases2. Neurological symptoms manifest in 15 to 20% of cases3.

Successful Treatment and Long-Term Follow-Up of Parry-Romberg Syndrome with Anterolateral Thigh Adipofascial Flap.

Anterolateral thigh adipofascial free flap transfer is a frequently used method to reconstruct facial symmetry and restore facial soft-tissue contour in patients with Parry-Romberg syndrome, but long-term prognosis and outcomes are unclear. The authors report their treatment experience with 42 patients between 2001 and 2017 using microsurgical free anterolateral thigh adipofascial flap transfer. Long-term follow-up results and final reconstructive outcomes were evaluated. A total of 42 patients were included. The follow-up ranged from 5 to 21 years. All patients were satisfied with the surgery. Photographic evaluation revealed significant enhancement of postoperative appearance. Numbness or hypesthesia of the local area was the most common symptom noted in long-term follow-up. This study evaluated the long-term results of treating Parry-Romberg disease with microsurgery using ALT free flap in the authors' department. Over 20 years of experience and the significant enhancement in overall appearance indicate a long-lasting, excellent outcome. Therapeutic, IV.

A case of progressive crossed hemiatrophy mistaken as panniculitis.

Progressive crossed hemiatrophy is an extremely rare clinical type of facial hemiatrophy that presents primarily as unilateral facial atrophy and contralateral trunk or limb involvement. The undistinguishable clinical manifestation and pathological changes complicate diagnosis, especially at the onset of the disease when presenting with less clinical evidence. Here, we report a case of a 9-year-old boy started with left scalp induration, following with subcutaneous tissues atrophy on the right trunk. He was mistaken as panniculitis based on the pathologic findings and treated with topical tacrolimus without any improvement. Immune-related tests were implemented to exclude connective tissues. Imaging examinations such as magnetic resonance was conducted to evaluate the range and degree of the involvement of the skin, soft tissue, and cranial changes. Although no effective treatment to hold back the progress has been reported so far, surgeries might work to restore the appearance to some extent or improve central nerves symptoms if they exist.

Treatment of Progressive Hemifacial Atrophy by Cartilage Graft and Free Adipofascial Flap Combined with Three-Dimensional Planning.

Progressive hemifacial atrophy (PHA) is a rare disease characterized by progressive atrophy of skin, soft tissue, muscles, and underlying bone structures. For severe PHA patients with obvious bone deformities, skeletal framework reconstruction is needed in addition to soft-tissue augmentation. The authors propose a new combinatorial surgical method using rib cartilage graft and free adipofascial flap for restoring facial symmetry. To improve the surgical accuracy, preoperative three-dimensional planning and printing was used. Twelve patients with severe facial atrophy were included in the authors' study. Three-dimensional facial image analyses were performed preoperatively to quantify the facial asymmetry. Rib cartilages were harvested and sculptured to the appropriate shape created by three-dimensional planning and fixed to the atrophic bone. The circumflex scapular artery-based adipofascial flap was transplanted to repair soft-tissue deficiency. A residual small monitor flap was left with the adipofascial flap. A revision surgery was performed to perfect the repair if the contour was suboptimal 6 months postoperatively. The adipofascial flaps survived in all 12 patients. All patients achieved good healing without complications. At 1 more year after surgery, the rib cartilage was still in position and rarely absorbed. The morphologic and volumetric difference between the affected side and the unaffected side was improved significantly postoperatively. All patients were satisfied with the results, and no more additional operations were required. The combinatorial surgery of rib cartilage graft and free adipofascial flap in the setting of three-dimensional planning and printing can be a good choice in restoring facial symmetry in severe cases of PHA. Therapeutic, IV.

A Case Report: Simultaneous Presentation of Morphea and Multiple Sclerosis, a Diagnostic and Management Challenge. (P8-3.018)

<h3>Objective:</h3> To describe a unique case of a patient with multiple sclerosis and concurrent morphea complicating the diagnostic and therapeutic picture. <h3>Background:</h3> Localized scleroderma, also called morphea, is an inflammatory skin condition causing excess collagen deposition and thickened skin. Parry-Romberg Syndrome is one subtype characterized by linear facial involvement. Rarely, neurologic symptoms manifest as seizures, neuropathy, CNS vasculitis, and white matter abnormalities on MRI. Prior case reports of CNS involvement are associated with T2 hyperintense lesions, predominantly subcortical and juxtacortical, ipsilateral to the cutaneous lesions and often localized to one brain area. Multiple sclerosis (MS) is a primary CNS demyelinating condition, most frequently with a relapsing remitting presentation. It is characterized by white matter abnormalities on MRI, usually with periventricular, juxtacortical, cortical or brainstem and spinal cord lesions. We present a 41-year-old African American female previously diagnosed with MS and started on ofatumumab. She presented in hopes of switching therapies due to injection fatigue. On exam, she exhibited left facial discoloration and hemifacial atrophy, progressively worsening for years. She was referred to a dermatologist and subsequently diagnosed with morphea via skin biopsy. MRI showed bihemispheric periventricular, subcortical, and juxtacortical demyelinating lesions and global atrophy. She also had cervical and thoracic spinal cord involvement. Imaging was more suggestive of MS than CNS morphea, and the concomitant diagnosis of both conditions was made. Given that none of the disease modifying therapies approved for MS are compatible with treatment of morphea, this presented a challenging scenario. The patient was continued on a B-cell therapy for MS and started on monthly methylprednisolone infusions for morphea, with clinical stability to date. <h3>Design/Methods:</h3> NA <h3>Results:</h3> NA <h3>Conclusions:</h3> We present a unique diagnostic dilemma of concomitant presentation of MS and morphea versus CNS involvement of morphea. Ultimately, the distinct imaging features of both disorders on MRI guided this patient’s diagnosis and treatment. <b>Disclosure:</b> Dr. Thakkar has nothing to disclose. Dr. Sharkus has nothing to disclose. Dr. McMahon has nothing to disclose. An immediate family member of Dr. Thon has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Horizon. An immediate family member of Dr. Thon has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. An immediate family member of Dr. Thon has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Genentech. Dr. Thon has received personal compensation in the range of $500-$4,999 for serving as a Focus group participant with Alexion. Olga Thon has nothing to disclose.

Linear Scleroderma en Coup de Sabre Presenting with Stroke and Seizure (P3-5.022)

<h3>Objective:</h3> To describe the neurologic complications and imaging findings in a patient with linear scleroderma <i>en coup de sabre</i> (LScs) and associated Parry-Romberg syndrome. <h3>Background:</h3> LScs is a rare subset of localized scleroderma. Once thought to be an exclusively cutaneous disorder, recent studies suggest that rheumatologic, ophthalmologic, and neurologic symptoms occur in up to 20% of cases. <h3>Design/Methods:</h3> Case report. <h3>Results:</h3> A 32-year-old woman with LScs, associated Parry-Romberg syndrome, migraine, and prior left hemispheric stroke thought to be secondary to vasculitis presented with right arm and leg numbness. She had left hemifacial atrophy. Brain MRI showed an acute ischemic stroke in the posterior limb of the left internal capsule and a chronic T2/FLAIR-hyperintensity in the left frontal lobe immediately beneath the cutaneous forehead and scalp scar. She was previously on mycophenolate for presumed vasculitis, however she had discontinued it due to concern about immunosuppression during the COVID-19 pandemic. Computed tomography angiography, digital subtraction angiography and lumbar puncture were normal. Her stroke was thought to be due to small vessel occlusion. The occurrence of two ischemic strokes ipsilateral to the cutaneous changes and facial atrophy, the ipsilateral MRI findings mirroring the <i>coup de sabre</i> scar, and the occurrence of the new stroke after discontinuation of mycophenolate together suggest an underlying vasculopathy/vasculitis associated with her scleroderma. Her hospital course was complicated by episodes of rapid rhythmic shaking of the right leg with no alteration of awareness, suggesting simple focal motor seizures. She was started on a prednisone with plans for steroid-sparing immunosuppressive therapy (rituximab and mycophenolate). On follow-up evaluation, she was recovering well. <h3>Conclusions:</h3> LScs and Parry-Romberg syndrome are associated with multiple neurologic complications including ischemic strokes, seizures, and headaches. This case highlights the underrecognized relationship between neurologic complications and LScs and suggests an underlying inflammatory process responsive to immunosuppressive therapy. <b>Disclosure:</b> Dr. Greige has nothing to disclose. Dr. Feske has received publishing royalties from a publication relating to health care.