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    Primary Sjögren's syndrome in patients with celiac disease.
    Turk J Med Sci 2017 Apr 18;47(2):430-434. Epub 2017 Apr 18.
    Division of Rheumatology and Immunology, Department of Internal Medicine, Akdeniz University Faculty of Medicine, Antalya, Turkey.
    Background/aim: Many autoimmune diseases occur concomitantly with celiac disease (CD). We aimed to determine the frequency of Sjögren's syndrome (SS) in CD patients based on SS-specific serology verified by minor labial salivary biopsy.

    Materials And Methods: Eight-two patients with CD were included in the study. Read More

    Th1- and Th17-polarized immune infiltrates in eosinophilic fasciitis - a potential marker for histopathologic distinction from morphea.
    J Cutan Pathol 2017 Apr 10. Epub 2017 Apr 10.
    Pathology Service, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA.
    Morphea (localized scleroderma) and eosinophilic fasciitis (EF) are rare fibrosing disorders which may present a diagnostic challenge. While histopathologic features are often distinct, in some cases there may be overlap. T-cells contribute to etiopathogenesis of both autoimmune conditions. Read More

    Erythema multiforme following pneumococcal vaccination.
    Acta Dermatovenerol Alp Pannonica Adriat 2017 Mar;26(1):25-26
    Department of Dermatology, University of Patras, Patras, Greece.
    Erythema multiforme (EM) is an acute and usually self-limited immune-mediated mucocutaneous disorder that is a hypersensitivity reaction to drugs, infections, and vaccines. Clinically, it is characterized by maculopapular, target-like lesions symmetrically distributed on the extremities (minor form) or additionally affecting one or more mucous membranes and causing epidermal detachment involving < 10% of the total body surface area (major form). We report a novel association between pneumococcal vaccination and the development of EM in a 2. Read More

    Pregnancy-associated morphea: a case report and literature review.
    Dermatol Online J 2017 Jan 15;23(1). Epub 2017 Jan 15.
    Department of Internal Medicine, Saint Vincent Hospital, Worcester, MA.
    Morphea, also known as localized scleroderma, is arare fibrosing disorder of the skin, the pathogenesisof which is incompletely understood. It is thought,however, to involve interplay of genetic dispositionand triggering environmental factors, such asinfections and autoimmunity. Pregnancy as a potentialtrigger has only been reported in four cases. Read More

    Morphea and Eosinophilic Fasciitis: An Update.
    Am J Clin Dermatol 2017 Mar 16. Epub 2017 Mar 16.
    Department of Dermatology, Radboud University Medical Centre, Nijmegen, The Netherlands.
    Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. Read More

    [Generalized circumscribed scleroderma with blisters].
    Hautarzt 2017 Mar 16. Epub 2017 Mar 16.
    Klinik für Dermatologie, Allergologie und Phlebologie, Klinikum Bremerhaven Reinkenheide, Postbrookstr. 103, 27574, Bremerhaven, Deutschland.
    The patient suffered from a 20-year course of generalized circumscribed scleroderma and presented with blisters in circumscribed areas of the affected skin. The development of subepidermal blisters has been described in all clinical forms of circumscribed scleroderma. Aetiology and pathogenesis of blister formation have not yet been clarified. Read More

    Histopathological changes in morphea and their clinical correlates: Results from the Morphea in Adults and Children Cohort V.
    J Am Acad Dermatol 2017 Mar 9. Epub 2017 Mar 9.
    Department of Dermatology University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address:
    Background: Histopathological features in morphea (localized scleroderma) and their clinical correlates are poorly described.

    Objective: We sought to systematically describe histologic changes of morphea in a large, well-annotated cohort and determine the association between histopathology and clinical features.

    Methods: This was a cross-sectional study of 83 patients enrolled in the Morphea in Adults and Children cohort. Read More

    Localized scleroderma: clinical and epidemiological features with emphasis on adulthood- versus childhood-onset disease differences.
    J Eur Acad Dermatol Venereol 2017 Mar 8. Epub 2017 Mar 8.
    Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
    Background: Localized scleroderma is a rare inflammatory skin disorder that affects the skin and sometimes underlying subcutaneous tissue, muscles or bones. The disease has two modes of onset: juvenile- (JLS) and adult-onset (aLoS). Clinical features have impact on diagnostic and treatment recommendations, but no consensus on the disease management depending on the age at diagnosis was given. Read More

    Increased expression of latent TGF-β-binding protein 4 affects the fibrotic process in scleroderma by TGF-β/SMAD signaling.
    Lab Invest 2017 Mar 6. Epub 2017 Mar 6.
    Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China.
    Scleroderma is a fibrosis-related disorder characterized by cutaneous and internal organ fibrosis, and excessive collagen deposition in extracellular matrix (ECM) is a major cause of fibrosis. Transforming growth factor-β (TGF-β)/SMAD signaling has a central role in the pathogenesis of fibrosis by inducing abnormal collagen accumulation in ECM, and latent TGF-β-binding protein 4 (LTBP-4) affects the secretion of latent TGF-β to ECM. A previous study indicated that bleomycin (BLM) treatment increased LTBP-4 expression in lung fibroblasts of Thy-1 knockout mice with lung fibrosis, and LTBP-4 further promoted TGF-β bioavailability as well as SMAD3 phosphorylation. Read More

    Clinical utility of hypo- and hyperpigmentation of skin in diffuse cutaneous systemic sclerosis.
    Int J Rheum Dis 2017 Mar 6. Epub 2017 Mar 6.
    Rheumatology Department, Waikato Hospital, Hamilton, New Zealand.
    Aim: Cutaneous involvement is an early manifestation of systemic sclerosis (SSc). Localized areas of 'salt and pepper skin' (S&P) may develop. We hypothesize that S&P skin occurs frequently in diffuse cutaneous (dc) SSc which can be used in its early diagnosis and may correlate with joint contractures. Read More

    Human endogenous retroviruses and chosen disease parameters in morphea.
    Postepy Dermatol Alergol 2017 Feb 7;34(1):47-51. Epub 2017 Feb 7.
    Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland.
    Introduction: Morphea (localized scleroderma) is a relatively rare disease characterized by excessive skin fibrosis. Human endogenous retroviruses (HERV) are largely distributed within the human genome with hundreds of thousands of elements. The HERV have been widely studied in autoimmune disorders, yet hardly ever assessed in diseases with a good prognosis such as morphea. Read More

    [Interferon in combined therapy of human papillomavirus infections associated with sexually transmitted infections. linical observation].
    Urologiia 2016 Nov(5):103-108
    Moscow Regional Clinical Center of Skin and Sexually-Transmitted Diseases, Moscow.
    To investigate the effectiveness of Ingaron (interferon-) in the treatment of HPV infection associated with sexually transmitted infections, the authors analyzed the scientific literature on the association of human papillomavirus infection with other viral and microbial pathogens. A clinical case of the association of human papillomavirus infection, urogenital infections (urogenital chlamydia and genital herpes) and localized scleroderma penis is described. The results of integrated therapy of diseases with the help of Interferon-gamma have been presented. Read More

    Successful infliximab therapy in a patient with comorbid spondyloarthritis, primary biliary cirrhosis and generalized morphea.
    Acta Clin Belg 2017 Feb 23:1-4. Epub 2017 Feb 23.
    a Faculty of Medicine, Department of Physical Medicine and Rehabilitation , Canakkale Onsekiz Mart University , Canakkale , Turkey.
    The patient in this report was diagnosed simultaneously with primary biliary cirrhosis (PBC), spondyloarthritis, and generalized morphea and was started on infliximab therapy. In addition to an improvement in clinical symptoms with this therapy, an improvement was also observed in laboratory parameters such as cholestatic enzymes, C-reactive protein, and erythrocyte sedimentation rate. Infliximab was well tolerated in this 56-year-old patient. Read More

    Multiplex assessment of serum cytokine and chemokine levels in idiopathic morphea and vitamin K1-induced morphea.
    Clin Rheumatol 2017 May 20;36(5):1173-1178. Epub 2017 Feb 20.
    Scleroderma Center and Jefferson Institute of Molecular Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
    The levels of 63 cytokines, chemokines, and growth factors were measured in the serum of four patients with idiopathic morphea and of one patient with vitamin K1-induced morphea employing a multiplex assay to identify the role of inflammatory/immunologic events in their pathogenesis. Full-thickness skin biopsies of affected skin were analyzed by histopathology. Luminex assays for 63 cytokines, chemokines, and growth factors were performed in the sera from four patients with idiopathic morphea and in two different samples of serum obtained in two separate occasions from one patient with vitamin K1-induced morphea. Read More

    Morphea "en coup de sabre": An unusual oral presentation.
    J Clin Exp Dent 2017 Feb 1;9(2):e315-e318. Epub 2017 Feb 1.
    DDS, MSc, Department of Oral Medicine and Oral Pathology, Universidad Andres Bello, Viña del Mar, Chile. Avenida Valparaíso 1560, Viña del Mar, Chile.
    Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Oral involvement is unusual and it may produce white linear fibrotic areas with a scar-like appearance, atrophy of tongue papillae, gingival recession and alveolar bone resorption. We report a case of a 13-year-old girl who consulted for progressive recession on the attached gingiva of her upper left incisors. Read More

    Sclerosing Dermatofibrosarcoma Protuberans Shows Significant Overlap With Sclerotic Fibroma in Both Routine and Immunohistochemical Analysis: A Potential Diagnostic Pitfall.
    Am J Dermatopathol 2017 Feb;39(2):83-88
    Departments of *Dermatology, and †Pathology, University of California San Francisco, San Francisco, CA.
    Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low-to-intermediate grade sarcoma with several histologic variants, including pigmented (Bednar tumor), sclerosing, myxoid, atrophic, and DFSP with fibrosarcomatous changes. Two patterns of sclerosis in DFSP can be observed, a sclerotic fibroma-like pattern and a morphea/lichen sclerosus-like pattern. Partial biopsies of sclerosing DFSPs with the sclerotic fibroma pattern can be misdiagnosed as sclerotic fibroma or other benign sclerosing tumors (eg, perineurioma, dermatofibroma). Read More

    Effects of UVA1 Phototherapy on Expression of Human Endogenous Retroviral Sequence (HERV)-K10 gag in Morphea: A Preliminary Study.
    Med Sci Monit 2017 Jan 28;23:505-512. Epub 2017 Jan 28.
    Department of Dermatology, Poznań University of Medical Sciences, Poznań, Poland.
    BACKGROUND Morphea, also known as localized scleroderma, is a rare autoimmune connective tissue disease characterized by skin fibrosis. UVA1 phototherapy is an important asset in the reduction of clinical manifestations in morphea. There are studies claiming that UV light modulates the expression of some human endogenous retroviral sequences. Read More

    Panniculitis: A summary.
    Semin Diagn Pathol 2016 Dec 27. Epub 2016 Dec 27.
    Section of Dermatopathology,Dermatopathology, Division of Surgical Pathology & Cytopathology, University of Virginia Medical Center, Charlottesville, VA, USA. Electronic address:
    The diagnosis of panniculitis is felt to be a confusing topic by some pathologists. This summarical article presents inflammatory diseases of the subcutis in a systematic fashion, based on whether they are centered on fibrovascular septa or the adipose lobules, and whether morphologic vasculitis is present or not. Septocentric, non-vasculitis disorders include erythema nodosum, panniculitis that follows the use of "biological" therapeutic agents, lipodermatosclerosis, post-irradiation panniculitis, morphea profunda, and necrobiosis lipodica profunda. Read More

    Pseudohypertension-Like Presentation: An Exceptionally Rare Presentation in an Athletic Female Patient with Morphea.
    Case Rep Dermatol Med 2016 29;2016:7027352. Epub 2016 Dec 29.
    Novel Psychoactive Substances Research Unit, University of Hertfordshire Doctoral College, Hertfordshire University, Hatfield, UK; Faculty of Medicine, University of Baghdad, Baghdad, Iraq.
    Introduction. Pseudohypertension is a condition which mainly occurs due to thickening-calcification of tunica intima of the arterial wall, leading to a faulty measurement of the intra-arterial blood pressure. To the best of our knowledge, this is the first case report in literature, of a pseudohypertension-like presentation in association with Morphea en plaque. Read More

    Musculoskeletal MRI findings of juvenile localized scleroderma.
    Pediatr Radiol 2017 Apr 14;47(4):442-449. Epub 2017 Jan 14.
    Nemours Children's Health System/Alfred I. duPont Hospital or Children, 1600 Rockland Road, Wilmington, DE, 19803, USA.
    Background: Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes.

    Objective: There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children. Read More

    Morphoea with prominent plasma cell endoneuritis.
    Clin Exp Dermatol 2017 Mar 4;42(2):196-199. Epub 2017 Jan 4.
    Department of Dermatopathology, St Thomas' Hospital, London, UK.
    Morphoea (localized scleroderma) is a cutaneous inflammatory condition characterized by the development of indurated and discoloured plaques. The histological features of morphoea typically include a superficial and deep perivascular and periadnexal chronic inflammatory infiltrate associated with variable degrees of dermal and/or subcutaneous sclerosis. The infiltrate is typically composed of lymphocytes, macrophages and conspicuous plasma cells. Read More

    Retinoic acid for treatment of systemic sclerosis and morphea: A literature review.
    Dermatol Ther 2017 Mar 29;30(2). Epub 2016 Dec 29.
    University of California, Los Angeles, Los Angeles, California, 90095.
    Systemic sclerosis and morphea are connective tissue diseases characterized by tightening, thickening, and hardening of the skin, leading to significant morbidity. Unfortunately, current treatment options have limited efficacy for many patients. Cutaneous manifestations of these diseases arise from excess collagen deposition and fibrosis in the skin, through pathogenic mechanisms which have yet to be extensively detailed at the causal immune and cellular levels. Read More

    Specific skin signs as a cutaneous marker of diabetes mellitus and the prediabetic state - a systematic review.
    Dan Med J 2017 Jan;64(1)
    Introduction: Diabetes mellitus and the prediabetic state are associated with a number of skin manifestations. This study is a systematic review of the following manifestations: acanthosis nigricans (AN), skin tags (ST), diabetic dermopathy (DD), rubeosis faciei (RF), pruritus (PR), granuloma annulare (GA), necrobiosis lipoidica (NL), scleroedema diabeticorum (SD) and bullosis diabeticorum (BD). These conditions possibly relate to underlying diabetogenic mechanisms. Read More

    Primary cutaneous anaplastic large cell lymphoma masquerading as large pyogenic granuloma.
    Indian Dermatol Online J 2016 Nov-Dec;7(6):526-528
    Department of Dermatology, Venereology and Leprosy, IGMC, Shimla, Himachal Pradesh, India.
    Primary cutaneous anaplastic large cell lymphoma (pcALCL) forms 9% of the cutaneous T-cell lymphomas. It usually presents as solitary reddish brown ulcerating nodule or indurated plaque. Sometimes, it mimics other dermatological diseases such as eczema, pyoderma gangrenosum, pyogenic granuloma, morphea, and squamous cell carcinoma. Read More

    Autoimmune collagen vascular diseases: Kids are not just little people.
    Clin Dermatol 2016 Nov - Dec;34(6):678-689. Epub 2016 Jul 9.
    Assistant Professor of Dermatology, University of Connecticut School of Medicine, Farmington, CT.
    Morphea, dermatomyositis (DM), and discoid lupus erythematosus (DLE) are autoimmune collagen vascular diseases that can present at any age. In all three of these diseases, the tenants of diagnosis and treatment are largely the same in both children and adults, with a few notable differences. Children with morphea are more likely to present with the linear subtype and have a higher incidence of extracutaneous manifestations. Read More

    The role of local temperature and other clinical characteristics of localized scleroderma as markers of disease activity.
    Int J Dermatol 2017 Jan;56(1):63-67
    Department of Pediatric Medicine, Dermatology Section, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
    Background: Determining the activity of localized scleroderma (LS) remains crucial for decision making, and reliable clinical indicators of activity are lacking. Our objective in this study was to analyze the utility of infrared thermography (IRT) in assessing the activity of LS according to existing clinical scales. A secondary objective was to study whether clinical characteristics of patients and/or IRT have any role in predicting the activity of LS. Read More

    Lacrimal Drainage System Involvement in Linear Scleroderma.
    Ophthal Plast Reconstr Surg 2016 Dec 7. Epub 2016 Dec 7.
    Govindram Seksaria Institute of Dacryology, L.V. Prasad Eye Institute, Hyderabad-34, India.
    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. Read More

    Scleroderma with Nodular Scleroderma.
    Case Rep Dermatol 2016 Sep-Dec;8(3):303-310. Epub 2016 Nov 14.
    Division of Dermatology, Department of Medicine, Phramongkutklao Hospital, Bangkok, Thailand.
    Background: Nodular scleroderma is a rare variant of scleroderma which can occur in connection with systemic sclerosis or morphea. A biopsy from the lesion can demonstrate the scleroderma pattern, i.e. Read More

    Disease Manifestation and Inflammatory Activity as Modulators of Th17/Treg Balance and RORC/FoxP3 Methylation in Systemic Sclerosis.
    Int Arch Allergy Immunol 2016 1;171(2):141-154. Epub 2016 Dec 1.
    Division of Pediatric Rheumatology, and Special Immunology, University Hospital Würzburg, University of Würzburg, Würzburg, Germany.
    Background: There is much evidence that T cells are strongly involved in the pathogenesis of localized and systemic forms of scleroderma (SSc). A dysbalance between FoxP3+ regulatory CD4+ T cells (Tregs) and inflammatory T-helper (Th) 17 cells has been suggested.

    Methods: The study aimed (1) to investigate the phenotypical and functional characteristics of Th17 and Tregs in SSc patients depending on disease manifestation (limited vs. Read More

    Histopathological and clinical traps in lichen sclerosus: a case report.
    Rom J Morphol Embryol 2016 ;57(2 Suppl):817-823
    Department of Ophthalmology, "Grigore T. Popa" University of Medicine and Pharmacy, Iassy, Romania;
    Lichen sclerosus et atrophicus and limited systemic scleroderma (acrosclerosis) are inflammatory skin diseases that ultimately evolve into two distinct modes of atrophic scar formation, but which can easily be confused clinically. They are very rarely associated. The literature has reported cases in which lichen sclerosus was associated with various forms of scleroderma, but often with localized morphea. Read More

    Radiation-induced lichen sclerosus of the vulva : First report in the medical literature.
    Wien Med Wochenschr 2017 Mar 10;167(3-4):74-77. Epub 2016 Nov 10.
    Department of Dermatology, University of Virginia Health System, Charlottesville, VA, USA.
    A 67-year-old woman presented with a firm plaque in the perineal region, 16 months after diagnosis of a high-grade basaloid squamous cell carcinoma of the vagina and treatment by external beam radiation therapy and vaginal cuff brachytherapy. The differential diagnosis included radiation-induced morphea, radiation dermatitis, or, possibly, radiation-induced lichen sclerosus. Biopsy findings, including special staining, confirmed the diagnosis of radiation-induced lichen sclerosus. Read More

    Linear Sclerodermoid Lupus Erythematosus Profundus in a Child.
    Am J Dermatopathol 2016 Dec;38(12):904-909
    *Ackerman Academy of Dermatopathology, New York, NY; †Department of Dermatology, Kasr Alainy Faculty of Medicine, Cairo University, Giza, Egypt; ‡Departments of Internal Medicine and Pathology and Laboratory Medicine, Texas A&M University Health Science Center, Dallas, TX; §Department of Pediatrics, Joseph M. Sanzari Children's Hospital, Hackensack University Medical Center, Hackensack, NJ; and ¶Department of Dermatology, New York Presbyterian Hospital, Columbia University Medical Center, New York, NY.
    Lupus erythematosus panniculitis, also known as lupus profundus, is a variant in the clinicopathological spectrum of lupus erythematosus (LE) affecting about 2%-3% of LE patients. A linear configuration of LE panniculitis has been reported rarely with rare reports describing the coexistence of different forms of cutaneous LE and localized morphea. In this study, the authors present a 9-year-old girl with linear arrangement of subcutaneous nodules on her left forearm. Read More

    Ophthalmological manifestations of Parry-Romberg syndrome.
    Surv Ophthalmol 2016 Nov - Dec;61(6):693-701. Epub 2016 Apr 1.
    Department of Ophthalmology, University of Cologne, Cologne, Germany.
    Parry-Romberg syndrome is a rare disease characterized by slowly progressive atrophy affecting facial subcutaneous tissues, including the underlying muscles and osteocartilaginous structures. Various periocular, ocular, and neuro-ophthalmological manifestations have been described in Parry-Romberg syndrome. The most common periocular disorders include enophthalmos, eyelid, and orbit alterations. Read More

    Sclerotic Regressing Large Congenital Nevus.
    Pediatr Dermatol 2016 Nov 25;33(6):e366-e367. Epub 2016 Oct 25.
    Second Department of Dermatology, Faculty of Medicine, Aristotle University, Papageorgiou General Hospital, Thessaloniki, Greece.
    Regression of congenital nevi is usually associated with loss of pigment or halo formation. In rare cases, regression is characterized by sclerosis and hair loss. We describe a rare case of a sclerotic hypopigmented large congenital melanocytic nevus in which a localized scleroderma-like reaction process of regression seemed to have started in utero and progressed throughout early childhood. Read More

    Scleroderma and dentistry: Two case reports.
    J Med Case Rep 2016 Oct 24;10(1):297. Epub 2016 Oct 24.
    General Practitioner, Wenatchee, Washington, USA.
    Background: Scleroderma is a chronic connective tissue disorder with unknown etiology. It is characterized by excessive deposition of extracellular matrix in the connective tissues causing vascular disturbances which can result in tissue hypoxia. These changes are manifested as atrophy of the skin and/or mucosa, subcutaneous tissue, muscles, and internal organs. Read More

    Early- and late-stage morphea subtypes with deep tissue involvement is treatable with Abatacept (Orencia).
    Semin Arthritis Rheum 2016 Sep 4. Epub 2016 Sep 4.
    Rheumatology Department, University Hospital Limerick, Dooradoyle, Limerick, Ireland; Graduate Entry Medical School, University of Limerick, Limerick, Ireland.
    Objectives: This case series explores the potential efficacy of Abatacept in patients presenting with morphea subtypes and deep tissue involvement.

    Methods: Three patients with established morphea subtypes and deep tissue involvement and with no contraindication to Abatacept were included in this prospective open-label study. The index patient was exceptionally severely affected with a mean Modified Rodnan Skin Score (MRSS) of 38/51. Read More

    The Chronic Encephalopathy of Parry Romberg Syndrome and En Coupe De Sabre with a 31-Year-History in a West Indian Woman: Clinical, Immunologic and Neuroimaging Abnormalities.
    Neurol Int 2016 Sep 30;8(3):6661. Epub 2016 Sep 30.
    University of the West Indies; Radiology Department, University of the West Indies, Trinidad and Tobago.
    We describe a case of Parry Romberg syndrome/en coupe de sabre in a woman whose disease started as seizures at age 8 but was diagnosed at the age 39. During these 31 years she got married, completed a first degree at university, had two successful pregnancies and has been gainfully employed. The features of generalized tonic-clonic seizures, autoimmune abnormalities, ocular abnormalities, morphea en coup de sabre and brain imaging abnormalities were present. Read More

    Nicolau syndrome and localized panniculitis: a report of dual diagnoses with an emphasis on morphea profunda-like changes following injection with glatiramer acetate.
    J Cutan Pathol 2016 Nov 8;43(11):1056-1061. Epub 2016 Sep 8.
    Department of Dermatology, Oregon Health and Sciences University, Portland, OR, USA.
    Glatiramer acetate, given as a 40 mg subcutaneous injection thrice weekly, was recently approved by the FDA based on data suggesting better compliance and a more favorable side effect profile compared to lower dose, daily dosing. The most commonly reported adverse events are transient injection site reactions involving redness and pain at the site; however, more pronounced panniculitis and lipoatrophy have also been reported. Here, we present the case of a 51-year-old female treated with higher dose glatiramer acetate who presented with a cutaneous injection site reaction consistent with Nicolau syndrome. Read More

    Antibodies against antigens related to Scleroderma in a cohort of patients with Morphea.
    G Ital Dermatol Venereol 2016 Oct 5. Epub 2016 Oct 5.
    Department of Dermatology, Spedali Civili and University of Brescia, Brescia, Italy -
    Background: Morphea is a rare fibrosing skin disorder. Antinuclear antibodies (ANA), antihistone and rheumatoid factor are detected in high rate of morphea cases. Scleroderma-related antibodies are usually absent. Read More

    Neurological abnormalities in localized scleroderma of the face and head: a case series study for evaluation of imaging findings and clinical course.
    Int J Neurosci 2016 Oct 26:1-5. Epub 2016 Oct 26.
    a Department of Dermatology, School of Medicine in Katowice , Medical University of Silesia , Katowice , Poland.
    Introduction: Localized scleroderma (LoS) of the face and head is often associated with neurological manifestations and/or imaging abnormalities in the central nervous system (CNS).

    Case Series: We present an analysis of 20 cases of LoS affecting the face and head. The CNS symptoms and/or abnormalities in high-resolution computed tomography (HRCT) and/or magnetic resonance imaging (MRI) were observed in 12 patients (60%). Read More

    Predictors of Longitudinal Quality of Life in Pediatric Localized Scleroderma.
    Arthritis Care Res (Hoboken) 2016 Oct 1. Epub 2016 Oct 1.
    Objective: Localized scleroderma (LS) can negatively affect children's quality of life (QoL), but predictors of impact are not well described. We sought to identify predictors of QoL impact in pediatric LS patients.

    Methods: We analyzed longitudinal data from a single-center cohort of pediatric LS patients, using hierarchical generalized linear modeling (HGLM) to identify predictors of QoL impact. Read More

    Esophageal abnormalities in juvenile localized scleroderma: is it associated with other extracutaneous manifestations?
    Rev Bras Reumatol 2016 Sep 17. Epub 2016 Sep 17.
    Universidade de São Paulo, Faculdade de Medicina, Unidade de Reumatologia Pediátrica, São Paulo, SP, Brasil; Universidade de São Paulo, Faculdade de Medicina, Divisão de Reumatologia, São Paulo, SP, Brasil. Electronic address:
    Objective: To assess esophageal involvement (EI) in juvenile localized scleroderma (JLS) population and the possible association between this gastrointestinal manifestation and demographic data, clinical features, laboratory exams, treatments and outcomes.

    Methods: For a period of 31 years, 5,881 patients with rheumatic diseases were followed in our Pediatric Rheumatology Division. EI was defined by the presence of symptoms (solid/liquid dysphagia, heartburn, esophageal regurgitation, nausea/vomiting and epigastralgia) and confirmed by at least one EI exam abnormality: barium contrast radiography, upper gastrointestinal endoscopy and 24-hour esophageal pH-monitoring. Read More

    Generalized Morphea following Radiotherapy for an Intracranial Tumor.
    Indian J Dermatol 2016 Sep-Oct;61(5):581
    Department of Dermatology and Venereology, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand, India.
    Morphea is a localized scleroderma variety which can be circumscribed or generalized and is characterized by sclerotic plaques developing on trunk and limbs. Surgery and radiation have been implicated as etiological factors for the development of morphea. Majority of the radiation-induced morphea cases have occurred in patients with breast cancer. Read More

    [Facial linear scleroderma associated with neurological abnormalities relating to microangiopathy].
    Ann Dermatol Venereol 2016 Dec 20;143(12):831-835. Epub 2016 Sep 20.
    Service dermatologie, hôpital Larrey, université Paul Sabatier, CHU, 24, chemin de Pouvourville, TSA 30030, 31059 Toulouse cedex 9, France.
    Background: Linear scleroderma is a fibrotic disease affecting the skin and sometimes the deeper tissues. We describe a case of scleroderma associated with neurological anomalies not previously reported in the literature.

    Patients And Methods: A 16-year-old male patient presented in 2009 for hemifacial linear scleroderma. Read More

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