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Exp Dermatol 2020 Jun 27. Epub 2020 Jun 27.

Department of Dermatology, University Medical Center Regensburg, Franz-Josef-Strauss Allee 11, D-93053, Regensburg, Germany.

Ultraviolet A (UVA )-phototherapy (spectral range 340-400 nm) is a well-established treatment option for various skin diseases such as localized scleroderma. Recent improvements ofconventional UVA -light sources (metal-halide or fluorescent lamps) have brought attention to a new Light Emitting Diodes (LED)-technology with remarkable advantages in handling and clinical routine. This study providesa preclinical histological and molecular evaluation of an LED-basedUVA -prototype with a narrower spectral range (360-400 nm) for treating localized scleroderma. Read More

Curr Rheumatol Rep 2020 Jun 26;22(8):45. Epub 2020 Jun 26.

Department of Woman's and Child's Health, University of Padua, Via Giustiniani 3, 35128, Padua, Italy.

Purpose Of Review: Treatment of scleroderma in children is challenging since little is known about its pathogenesis. Herein, we review the most recent evidence regarding the treatment of juvenile scleroderma.

Recent Findings: According to the recent recommendations for Pediatric Rheumatology in Europe (SHARE), systemic treatment in localized scleroderma is needed when there is a risk for disability, such as in generalized or pansclerotic morphea and progressive linear scleroderma. Read More

Pediatr Int 2020 Jun;62(6):759-761

Department of Dermatology, University General Hospital of Patras, Rio, Greece.

Australas J Dermatol 2020 Jun 22. Epub 2020 Jun 22.

Department of Dermatology, Concord Repatriation General Hospital, Sydney, Australia.

J Am Acad Dermatol 2020 Jun 15. Epub 2020 Jun 15.

Merck & Co., Inc., Kenilworth, NJ, USA. Electronic address:

Cureus 2020 May 14;12(5):e8117. Epub 2020 May 14.

Family Medicine, Southern Illinois University School of Medicine, Springfield, USA.

Morphea, also known as localized scleroderma, is an uncommon idiopathic inflammatory disorder leading to the development of sclerotic plaques in the skin. The disorder preferentially affects females. The pathogenesis of morphea is not well-understood. Read More

Cureus 2020 May 12;12(5):e8067. Epub 2020 May 12.

Infectious Diseases, Northwell Health, Manhasset, USA.

Morphea or localized scleroderma is reported to be triggered through diverse stimuli. We present a case of morphea that presented as a non-healing wound with superimposed methicillin-sensitive (MSSA) infection. In our case, morphea was thought to have been potentially triggered by a post-surgical infection. Read More

J Rheumatol 2020 Jun 15. Epub 2020 Jun 15.

From the Division of Dermatology, Department of Medicine, Section of Community Pediatrics, Section of Pediatric Rheumatology, Department of Pediatrics, Dermatology Research Institute, University of Calgary, Calgary, Alberta, Canada. Address correspondence to Dr. N.J. Luca, ACH C4-348, Alberta Children's Hospital, 28 Oki Drive, Calgary, Alberta T3B 6A8, Canada. E-mail:

Brain magnetic resonance imaging (MRI) findings in craniofacial morphea including en coup de sabre (ECDS) localized scleroderma and Parry-Romberg syndrome (PRS; progressive facial hemiatrophy) are not unusual, but the relevance of rare abnormalities is unknown. We report 2 cases in which distant cerebellar lesions on brain MRI occurred with varying clinical neurologic manifestations. Read More

Autoimmun Rev 2020 Jun 12:102596. Epub 2020 Jun 12.

Regional Center of Pharmacovigilance, Department of Pharmacology, Cochin Hospital, Paris, France.

Introduction: Immune checkpoint inhibitors (ICIs) are associated with immune-related adverse events (irAEs). Among them, ICIs-induced systemic sclerosis (SSc) is poorly known.

Methods: To better characterize this irAE, our comprehensive approach combined the description of ICIs-induced scleroderma cases, the systematic review of the literature and the analysis of VigiBase, the WHO pharmacovigilance database. Read More

Hautarzt 2020 Jun 12. Epub 2020 Jun 12.

Hautklinik, Universitätsklinikum Heidelberg, Heidelberg, Deutschland.

We present a rare case of a 4-year-old girl suffering from a sclerotic lesion on the forehead as well as uveitis and epileptic seizures. The patient was diagnosed with linear scleroderma "en coup de sabre" (LSECDS). Faced with no gold standard for treatment of LSECDS with severe extracutaneous manifestations, the case was intensively discussed within an interdisciplinary team setting and successfully treated. Read More

J Eur Acad Dermatol Venereol 2020 Jun 12. Epub 2020 Jun 12.

Department of Dermatology, Tufts Medical Center, Tufts University School of Medicine, Boston, MA, United States.

Background: Hidradenitis suppurativa (HS) has been associated with auto-inflammatory conditions, yet the risk of developing connective tissue disease (CTD), morphea, and systemic vasculitis has not been well-characterized.

Objectives: We sought to evaluate the risk of developing CTD, morphea and systemic vasculitis in patients with HS.

Methods: Using claims data we identified patients with HS and used 2:1 risk-set sampling to identify patients without HS. Read More

J Child Neurol 2020 Jun 12:883073820931253. Epub 2020 Jun 12.

Division of Pediatric Rheumatology, Department of Pediatrics, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.

Objective: Parry-Romberg syndrome (PRS) and en coup de sabre (ECDS) are subtypes of craniofacial localized scleroderma. Systematic analyses of central nervous system imaging findings and their clinical associations in children are lacking. Here, we aim to characterize neuroimaging findings and associated neurological symptoms in these conditions. Read More

Semin Arthritis Rheum 2020 May 19;50(4):645-656. Epub 2020 May 19.

Department of Pediatrics, University of New Mexico, 1127 University Blvd NE, MSC10, Albuquerque, NM 87131-0001, USA; Department of Dermatology, University of New Mexico, 1127 University Blvd NE, MSC10, Albuquerque, NM 87131-0001, USA.

Background: Localized scleroderma (LS) is a rare chronic immune-mediated skin condition of unknown etiology characterized by an inflammatory response in the skin and subcutaneous tissues resulting in collagen deposition and subsequent fibrosis. There is no cure for LS. No therapies have been licensed specifically for the treatment of LS and the clinical management of the disease remains largely empirical. Read More

J Dermatolog Treat 2020 Jun 9:1-2. Epub 2020 Jun 9.

Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Indian Dermatol Online J 2020 Mar-Apr;11(2):288-290. Epub 2020 Mar 9.

Department of Dermatology, Venereology, and Leprosy, Dr. KN Barua Institute of Dermatological Sciences, Guwahati, Assam, India.

A 60-year-old male patient presented with a solitary pink plaque over the inner aspect of the left forearm of 4 years duration. The lesion was persistent and unresponsive to treatment. A skin biopsy was done with differential diagnosis of lupus vulgaris, tuberculoid leprosy, and granuloma annulare. Read More

Indian Dermatol Online J 2020 Mar-Apr;11(2):135-145. Epub 2020 Mar 9.

Department of Child Health, Christian Medical College, Vellore, Tamil Nadu, India.

Juvenile localized scleroderma (morphea) is the predominant scleroderma in childhood which affects the skin and may extend to the underlying fascia, muscle, joints and bone. The assessment of activity and damage can be done with a validated instrument like LoSCAT. Disease classified as "low severity" which includes superficial plaque morphea can be treated with topical mid potent- potent steroids, tacrolimus, calcipotriol or imiquimod in combination with phototherapy. Read More

Clin Case Rep 2020 May 9;8(5):837-839. Epub 2020 Mar 9.

Skin Research Center Shahid Beheshti University of Medical Sciences Tehran Iran.

Nodular or keloidal morphea, also known as nodular scleroderma, is a rare form of localized sclerosis (SSc) or morphea. In this paper, we reported a case of this rare entity with a review of the literature. Read More

Clin Exp Dermatol 2020 May 30. Epub 2020 May 30.

Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Morphoea, also known as localized scleroderma, is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Paediatric-onset disease is not uncommon and is associated with frequent relapses. The disease has complex pathogenetic mechanisms and multiple clinical subtypes, and affects children of all ages. Read More

J Affect Disord 2020 May 25;274:334-338. Epub 2020 May 25.

School of Medicine, National Yang-Ming University, Taipei, Taiwan; Department of Psychiatry, Taipei Veterans General Hospital, Taipei, Taiwan. Electronic address:

Background: Major depressive disorder (MDD) has been implicated as a risk factor for various immune-related disorders; however, the association between MDD and subsequent autoimmune skin diseases (ASDs) remains unclear. This study aimed to investigate the association of MDD with risk of subsequent ASDs.

Methods: Subjects were recruited from the National Health Insurance Research Database in Taiwan. Read More

Clin Exp Dermatol 2020 May 24. Epub 2020 May 24.

Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Paediatric morphoea is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Defining optimum management strategies in paediatric morphoea remains an ongoing challenge, owing to the varied presentations and a relative paucity of paediatric-specific studies. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Read More

Dermatol Ther 2020 May 22:e13641. Epub 2020 May 22.

Department of Rheumatology, PLA General Hospital, Beijing, China.

Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by increased peripheral blood eosinophils and diffuse fasciitis, generalized morphea (GM) is a subtype of localized scleroderma, and IgA nephropathy is a chronic glomerulonephritis caused by abnormal deposition of IgA in the mesangial area of the glomeruli. We describe a 49-year-old male patient with hard skin, cutaneous hyperpigmentation, and proteinuria. The patient had suffered from a long disease course of hard skin, while urine protein was newly detected. Read More

Arthritis Care Res (Hoboken) 2020 May 20. Epub 2020 May 20.

Pediatric Rheumatology Unit, Department of Woman and Child Health, University of Padova, Italy, Padova.

Objective: to study disease course and long-term outcome of children with Linear Scleroderma (LiS) treated with methotrexate (MTX) since diagnosis.

Methods: Retrospective and cross-sectional study including consecutive children with LiS treated with MTX for >1 year and with at least 2 years follow-up. Disease course was analyzed by number of relapses and treatment changes. Read More

Medwave 2020 Apr 29;20(3):e7880. Epub 2020 Apr 29.

Escuela de Medicina Humana, Universidad Privada de Tacna, Tacna, Perú.

Progressive hemifacial atrophyor Parry-Romberg syndromeis a rare disease, classified as one of the forms of localized morphea or scleroderma. Its cause is unknown. It is characterized by atrophy of the skin, fat, muscles and underlying osteocartilaginous structures that usually affects the face and neck unilaterally, and is associated with neurological symptoms (secondary epilepsy) and involvement of other organs and systems. Read More

Clin Rheumatol 2020 May 18. Epub 2020 May 18.

Nucleus of Autoimmune Disease, Center Paranaense de Estudos em Dermatologia and Laboratorio Paulista de Dermatologia, Hospital BPMirante, Sao Paulo, Brazil.

J Dtsch Dermatol Ges 2020 May;18(5):447-454

Department of Dermatology, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.

Background And Objectives: Histopathologic diagnosis of fluoroscopy-induced radiation ulcer (FIRU) can be challenging if the past history of radiation exposure is unknown. Morphea is the most important differential diagnosis. This study was intended to identify clinical and pathologic features that can be used to distinguish FIRU from morphea. Read More

Australas J Dermatol 2020 May 14. Epub 2020 May 14.

Department of Dermatology, University Hospitals of Derby and Burton NHS Foundation Trust, Derby, UK.

Am J Clin Dermatol 2020 May 14. Epub 2020 May 14.

Department of Radiation Oncology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

Radiation therapy is a mainstay for the treatment of primary malignancies and metastatic disease and is associated with several dermatological adverse events that are underreported in the literature. The objective of this paper was to review the literature regarding cutaneous manifestations associated with radiation therapy in order to promote awareness of the cutaneous radiation therapy-associated adverse effects. This extensive literature review was performed using the Pubmed and Embase databases. Read More

Health Qual Life Outcomes 2020 May 12;18(1):133. Epub 2020 May 12.

Medical University of Silesia, Chair and Department of Dermatology, School of Medicine in Katowice, Francuska 20/24, 40-027, Katowice, Poland.

Background: Localized Scleroderma (LoS) is an autoimmune connective tissue disease that affects skin and less commonly subcutaneous tissues. The illness occurs in children and adults, and may have a serious impact on health-related quality of life (HRQoL). The goal of this study was to explore what factors might deteriorate scores on HRQoL measures in adult LoS patients. Read More

J Dermatol 2020 May 8. Epub 2020 May 8.

Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.

Ultraviolet (UV)A1 phototherapy is effective for T-cell-mediated skin diseases such as atopic dermatitis and mast cell-mediated skin diseases such as mastocytoma. UVA1 phototherapy is also effective against the sclerotic lesions of systemic sclerosis and morphea. Currently, in Japan, access to UVA1 phototherapy is limited because the UVA1 phototherapy device has not yet been approved. Read More

Cureus 2020 Apr 6;12(4):e7562. Epub 2020 Apr 6.

Department of Dermatology, University of Nevada Las Vegas School of Medicine, Las Vegas, USA.

Cutaneous sclerosis occurs in association with a variety of systemic diseases, including hematologic malignancy, plasma cell dyscrasias, solid organ tumors, and other systemic autoimmune conditions. Herein, we present a unique case of morphea/lichen sclerosus overlap arising in association with aplastic anemia. To expand upon this rare case, we also review the literature surrounding paraneoplastic sclerosing skin disorders. Read More

J Dtsch Dermatol Ges 2020 May 4. Epub 2020 May 4.

Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Würzburg, Germany.

Scleroderma is a heterogeneous group of fibrosing connective tissue disorders of unknown etiology. Morphea is a localized form of scleroderma that occasionally leads to chronic erosions and ulcerations of the skin. Fibrosis, inflammation and chronic ulcerations may eventually promote skin neoplasms; morphea is therefore a rare but established risk factor for cutaneous squamous cell carcinoma (cSCC). Read More

J Am Acad Dermatol 2020 Apr 28. Epub 2020 Apr 28.

Department of Dermatology, Boston University School of Medicine, Boston, MA. Electronic address:

Unstructured Abstract: Morphea and systemic sclerosis are inflammatory, sclerosing disorders. Morphea primarily affects the dermis and subcutaneous fat, while systemic sclerosis typically involves the skin and internal organs. Functional impairment and cosmetic disfigurement are common in both diseases. Read More

J Cutan Pathol 2020 Apr 12. Epub 2020 Apr 12.

Department of Dermatology, University of California, San Francisco, California, USA.

Background: Granuloma annulare (GA) is a skin disorder of uncertain etiology. Patch (type) GA is an uncommon variant of GA with a paucity of data characterizing it. We describe the features of 23 cases of patch GA. Read More

Indian J Dermatol Venereol Leprol 2020 Jul-Aug;86(4):359-365

Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Skin and Immune Diseases, Beijing, China.

Background: Linear cutaneous lupus erythematosus is a rare subtype of lupus erythematosus (LE) that develops linear lesions following the lines of Blaschko. Linear cutaneous lupus erythematosus may present as various subtypes of LE, including linear discoid lupus erythematosus. There are few reports about pigmentedlinear discoid lupus erythematosus in the literature. Read More

Biomed Res Int 2020 13;2020:9186274. Epub 2020 Mar 13.

Department of Dermatology, University of Rzeszow, Rzeszów, Poland.

Objective: The aim of the study was to evaluate QoL in patients suffering from morphea. . Sixty-five patients with morphea were recruited into this cross-sectional, prospective parallel study. Read More

Acta Ortop Mex 2019 Jul-Aug;33(4):261-264

Unidad Médica de Alta Especialidad (UMAE), Hospital de Traumatología y Ortopedia Lomas Verdes. Instituto Mexicano del Seguro Social (IMSS). Estado de México. México.

Introduction: Localized scleroderma is the most common form of sclerosis in children; it has an incidence of 2.7 per 100,000 inhabitants, with a predilection for the Caucasian and female races of 2.4 to 4. Read More

Dermatol Online J 2020 Feb 15;26(2). Epub 2020 Feb 15.

Department of Dermatology, Fukushima Medical University, Fukushima.

A 60-year old man developed skin hardening and edema on his extremities. Although he had been treated with oral prednisolone at another hospital, skin stiffness relapsed during tapering of prednisolone. At the initial visit to our department, physical examination showed skin hardening of the extremities and also symmetric erythematous macules on the back. Read More

JAMA Dermatol 2020 Apr 1. Epub 2020 Apr 1.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas.

JAMA Dermatol 2020 Apr 1. Epub 2020 Apr 1.

Department of Dermatology, UT Southwestern Medical Center, Dallas, Texas.

Importance: Prospective studies of the disease course in patients with morphea are lacking, particularly those comparing adults and children.

Objective: To investigate the disease course in patients with morphea treated with standard-of-care therapy using validated clinical outcome measures.

Design, Setting, And Participants: Prospective cohort study of 130 adults and children from the Morphea in Adults and Children cohort with at least 2 years of clinical follow-up and Localized Scleroderma Cutaneous Assessment Tool scores recorded at each study visit. Read More

JAMA Dermatol 2020 Apr 1. Epub 2020 Apr 1.

Department of Dermatology, Brigham and Women's Hospital, Boston, Massachusetts.

Importance: First-line systemic therapy for morphea includes methotrexate with or without systemic corticosteroids. When this regimen is ineffective, not tolerated, or contraindicated, a trial of mycophenolate mofetil (MMF) or mycophenolic acid (MPA)-referred to herein as mycophenolate-is recommended; however, evidence to support this recommendation remains weak.

Objective: To evaluate the effectiveness and tolerability of mycophenolate for the treatment of morphea. Read More

JAMA Dermatol 2020 Apr 1. Epub 2020 Apr 1.

Department of Dermatology, University of California, San Francisco.

Ann Hematol 2020 Mar 27. Epub 2020 Mar 27.

Faculty of Medicine, Department of Dermatology, Dokuz Eylul University, 35340 Inciraltı, Izmir, Turkey.

Clin Exp Dermatol 2020 Jul 22;45(5):632-634. Epub 2020 Apr 22.

Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan.

J Clin Med 2020 Mar 11;9(3). Epub 2020 Mar 11.

Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Francuska 20/24, 40-027 Katowice, Poland.

Localized scleroderma (LoS) is a chronic fibrosing disorder of the skin and, less commonly, subcutaneous tissues. As the disease causes subjective symptoms, cosmetic defects, and, at times, functional disability, subjects with LoS experience deterioration of their health-related quality of life (HRQoL). The influence of disease activity/severity and damage status on HRQoL measures in patients with LoS is scarcely known. Read More

Am J Dermatopathol 2020 Mar 6. Epub 2020 Mar 6.

Departments of Pathology, and.

Keloidal morphea is a rare variant of scleroderma, which often can be clinically confused with keloid or scar formation. We report a 34-year-old woman with a medical history of asthma and Raynaud's phenomenon, presented for the evaluation and management of multiple erythematous hyperpigmented annular plaques reportedly developed after taking trimethoprim/sulfamethoxazole. An initial skin biopsy showed findings supportive of a drug eruption. Read More

Best Pract Res Clin Rheumatol 2020 Feb 5;34(1):101489. Epub 2020 Mar 5.

Centre for Rheumatology, Royal Free Campus, University College London, Rowland Hill Street, London NW3 2PF, UK. Electronic address:

Systemic sclerosis is a severe immune-mediated rheumatic disease by virtue of its clinical impact and mortality. There are a number of other sclerosing skin diseases that should be considered in the differential diagnosis and these are important because they may require specialist investigation and management. In addition, long-term follow up of the different conditions should reflect the risk of associated complications and anticipated duration of therapy. Read More

Eur Radiol 2020 Jul 6;30(7):4091-4097. Epub 2020 Mar 6.

Department of Medicine - DIMED, Radiology Institute, University of Padova, Via Giustiniani 2, 35100, Padua, Italy.

Objectives: Juvenile localized scleroderma (JLS) is a rare chronic autoimmune disease which can also affect bones and muscles. Nevertheless, muscle loss was not previously investigated in patients with JLS. Thus, the aim of this study was to retrospectively evaluate deep involvement and assess and quantify sarcopenia in JLS patients using magnetic resonance imaging (MRI). Read More

Gac Med Mex 2019 ;155(5):483-491

Instituto Nacional de Pediatría, Dermatology Department, Mexico City, Mexico.

Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. Read More

JAAD Case Rep 2020 Mar 12;6(3):166-168. Epub 2020 Feb 12.

Weill Cornell Medicine Department of Pathology and Laboratory Medicine, Division of Dermatopathology, New York, New York.

J Clin Aesthet Dermatol 2020 Jan 1;13(1):38-40. Epub 2020 Jan 1.

All authors are with the Department of Dermatology and Cutaneous Surgery at the University of Miami, Miller School of Medicine in Miami, Florida.

Oral mucosal involvement in patients with morphea is quite rare. Unilateral nevoid telangiectasia is a rare vascular dermatosis characterized by unilateral telangiectasia distributed in a dermatomal pattern, especially on the upper trunk and extremities. We describe a case of a 10-year-old female patient that presents with morphea on her left chin and upper lip extending to the inner oral mucosa as well as a unilateral nevoid telangiectasia on her left cheek as an early presentation of localized scleroderma. Read More