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    Pathophysiological Mechanisms in Sclerosing Skin Diseases.
    Front Med (Lausanne) 2017 18;4:120. Epub 2017 Aug 18.
    Department of Dermatology, University of Cologne, Cologne, Germany.
    Sclerosing skin diseases represent a large number of distinct disease entities, which include systemic sclerosis, localized scleroderma, and scleredema adultorum. These pathologies have a common clinical appearance and share histological features. However, the specific interplay between cytokines and growth factors, which activate different mesenchymal cell populations and production of different extracellular matrix components, determines the biomechanical properties of the skin and the clinical features of each disease. Read More

    European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis.
    J Eur Acad Dermatol Venereol 2017 Oct 8;31(10):1581-1594. Epub 2017 Aug 8.
    Department of Dermatology and Venereology, University Hospital of Cologne, Cologne, Germany.
    The term 'sclerosing diseases of the skin' comprises specific dermatological entities which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. Read More

    Implementation and evaluation of a rehabilitation concept in a patient suffering from Scleredema Adultorum Buschke: a case report.
    Disabil Rehabil 2017 Jul 24:1-3. Epub 2017 Jul 24.
    a Department of Physical Medicine, Rehabilitation and Occupational Medicine , Medical University of Vienna , Vienna , Austria.
    Purpose: Scleredema Adultorum Buschke is a disorder manifesting indurations of the skin mostly followed by musculoskeletal impairment. Data regarding this fact are seldom found and documentation of functional outcome of physical therapies and modalities related to Scleredema Adultorum Buschke is fragmentary. The aim of this case report is to demonstrate and to document an effective concept of rehabilitation in a patient suffering from Scleredema Adultorum Buschke. Read More

    Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.
    Clin Rev Allergy Immunol 2017 Jul 16. Epub 2017 Jul 16.
    Section of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.
    Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. Read More

    Scleroderma-like Disorders.
    Curr Rheumatol Rev 2017 06 11. Epub 2017 Jun 11.
    Fortis Escorts Hospital, Jaipur, India
    Scleroderma is a term used to describe diseases that involve hardening and tightening of the skin and the underlying subcutaneous connective tissue. It could be localized to skin and subcutaneous tissue, or may involve the internal organs too in systemic sclerosis. There are disorders that can cause hardening and tightening of skin and mimic scleroderma but are rarely associated with Raynaud phenomenon, sclerodactyly, and autoantibodies in the serum, features specific to scleroderma/systemic sclerosis. Read More

    Successful treatment with IVIg therapy of diabetes-associated scleredema severe progressive case and review of the literature.
    Dermatol Ther 2017 Jul 2;30(4). Epub 2017 Jun 2.
    Department of Medicine, University of Central Florida, Orlando, Florida.
    Scleredema is a deposition disorder which presents as diffuse, symmetric, non-pitting skin induration from dermal deposits of glycosaminoglycans ("mucins"). It classically affects the upper back and posterior neck, often causing skin tightness and decreased range of motion. In most patients the clinical course is chronic and treatment options are limited. Read More

    Scleroderma Mimickers.
    Curr Treatm Opt Rheumatol 2016 Mar 5;2(1):69-84. Epub 2016 Feb 5.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
    Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin involvement, presence or absence of systemic complications and unique associated laboratory abnormalities often help to distinguish between these diseases. It is imperative that an effort is made to accurately differentiate between scleroderma and its mimics, in order to guide long-term management and facilitate implementation of the appropriate treatment modality where indicated. Read More

    Cutaneous Manifestations of Diabetes Mellitus: A Review.
    Am J Clin Dermatol 2017 Aug;18(4):541-553
    Department of Dermatology, University Hospital Jena, Erfurter Straße 35, 07740, Jena, Germany.
    Diabetes mellitus is a widespread endocrine disease with severe impact on health systems worldwide. Increased serum glucose causes damage to a wide range of cell types, including endothelial cells, neurons, and renal cells, but also keratinocytes and fibroblasts. Skin disorders can be found in about one third of all people with diabetes and frequently occur before the diagnosis, thus playing an important role in the initial recognition of underlying disease. Read More

    Therapy resistant idiopathic scleredema: an underlying pathology not always present.
    Eur J Rheumatol 2015 Dec 20;2(4):163-164. Epub 2015 May 20.
    Department of Internal Medicine, Division of Rheumatology, Ege University Faculty of Medicine, İzmir, Turkey.
    Scleredema is a rare connective tissue disorder of unknown pathogenesis. Three types of scleredema have been described, based on its association with postinfection, monoclonal gammopathy and diabetes mellitus. We report herein a case of scleredema which the diagnosis didn't get specified. Read More

    Atypical Scleredema Involving the Hands in an Adolescent.
    Pediatr Dermatol 2016 Nov 30;33(6):e342-e343. Epub 2016 Aug 30.
    Division of Dermatology, Medical College of Georgia at Augusta University, Augusta, GA.
    Scleredema is a rare and incompletely understood disorder characterized by progressive skin thickening and induration typically affecting the trunk, neck, and proximal upper extremities. Hand and finger involvement is exceptionally rare, having been reported in only two infection-related cases. We present an atypical case of postinfectious scleredema involving the hands and discuss scleredema as an important potential cause of hand tightness and induration in adolescents. Read More

    [Risk factors for concurrent sepsis in neonates with necrotizing enterocolitis].
    Zhongguo Dang Dai Er Ke Za Zhi 2016 Aug;18(8):677-82
    Neonatal Diagnosis and Treatment Center, Children's Hospital of Chongqing Medical University/National Demonstration Base of Standardized Training Base for Resident Physicians/Ministry of Education Key Laboratory of Child Development and Disorders/Key Laboratory of Pediatrics in Chongqing/Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Chongqing 400014, China.
    Objective: To investigate the risk factors for concurrent sepsis in neonates with necrotizing enterocolitis (NEC).

    Methods: A retrospective analysis was performed for the clinical data of 273 neonates with NEC. The risk factors for concurrent sepsis were analyzed from the aspects of perinatal factors and treatment regimen before the diagnosis of NEC. Read More

    Scleredema of Buschke associated with difficult-to-control type 2 diabetes mellitus.
    Rev Assoc Med Bras (1992) 2016 May-Jun;62(3):199-201
    MSc in Anatomical Pathology from UFF. Adjunct Professor, Pathology, UFF, Niterói, RJ, Brazil.
    Scleredema of Buschke (SB) is a rare disorder of connective tissue characterized by diffuse non-pitting induration of the skin, mainly on the cervical, deltoid and dorsal regions. It is a cutaneous mucinosis of unknown etiology and is associated with bacterial or viral infections, hematological disorders and diabetes mellitus. Histopathological examination shows thickened dermis with wide collagen bundles separated by gaps that correspond to mucopolysaccharide deposits, visualized using special staining. Read More

    [Scleroderma-like disorders].
    Rev Med Interne 2016 Sep 3;37(9):616-24. Epub 2016 Feb 3.
    Service de médecine interne, hôpital Robert-Bisson, 4, rue Roger-Aini, 14100 Lisieux, France.
    The finding of hardening and thickening of the skin is common and can be encountered in immune mediated, metabolic, neoplastic, toxic, genetic diseases, or associated with protein deposits. The lack of Raynaud's phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies should question the diagnosis of scleroderma and trigger the search for a scleroderma-like disorder, for which treatment and prognosis differ. This article gives a review of these disorders and their main characteristics. Read More

    [Monoclonal immunoglobulin (M-Ig) and skin diseases from the group of mucinoses--scleredema adultorum Buschke and scleromyxedema. Description of four cases and an overview of therapies].
    Vnitr Lek 2015 Dec;61(12):1072-87
    Introduction: The mucinoses of the type of scleredema and scleromyxedema are diseases marked by excessive production of mucin deposits in the skin and subcutaneous tissue, which causes skin hardening. The skin and subcutaneous deposits hamper the movement of limbs, the thorax as well as mouth. The same mechanism also damages other organs (the heart, lungs, oesophagus). Read More

    Idiopathic scleredema.
    Dermatol Online J 2016 Dec 15;22(12). Epub 2016 Dec 15.
    Ronald O. Perelman Department of Dermatology, NYU School of Medicine, NYU Langone Medical Center.
    Scleredema, which also is known as scleredemaadultorum of Buschke, is an uncommonsclerodermiform condition that is characterizedby progressive thickening and hardening of theskin due to excessive dermal mucin and collagendeposition. The clinical course is variable, andprogression of disease may lead to functionalimpairment with limitations in mobility. The etiologyand pathogenesis are unknown although severalwell-known associations include streptococcalinfection; diabetes mellitus, particularly withmetabolic syndrome; and monoclonal gammopathy. Read More

    Long-term benefit of electron beam radiation therapy in the treatment of scleredema of Buschke.
    Adv Radiat Oncol 2016 Jan-Mar;1(1):21-25. Epub 2015 Dec 9.
    Department of Radiation Oncology, Division of Dermatology, University of Arizona, Tucson, Arizona.
    Introduction: Scleredema of Buschke is a rare connective tissue disorder presenting with woody thickening and induration of the nuchal and shoulder regions resulting in progressive decrease in the range of motion of the neck. Treatment options include several forms of systemic therapy with variable results. Local radiation therapy (RT) is often thought of as a secondary form of therapy. Read More

    Prevalence of Skin Changes in Diabetes Mellitus and its Correlation with Internal Diseases: A Single Center Observational Study.
    Indian J Dermatol 2015 Sep-Oct;60(5):465-9
    Department of Medicine, Sanjiban Hospital, Kolkata, India.
    Background And Aim: This single-center observational cross-sectional study has been done in an attempt to find out the prevalence of various skin manifestations in diabetes patients (DM) and their correlation with diabetes control and complications.

    Materials And Methods: Skin manifestations present over 12 months among those attend diabetes clinic were included in the study. Apart from demographic data and type, patients were also screened for micro vascular complications and control of diabetes over last 3 months. Read More

    Scleredema Diabeticorum - A Case Report.
    Mymensingh Med J 2015 Jul;24(3):606-9
    Dr Md Nahiduzzamane Shazzad, Medical Officer, Department of Rheumatology, Bangabandhu Sheikh Mujib MedicalUniversity (BSMMU), Dhaka, Bangladesh.
    Buschke Scleredema is a rare connective tissue disorder of unknown aetiology and characterized by thickening of the dermis. It resembles systemic sclerosis, eosinophilic fasciitis and cutaneous amyloidosis. Scleredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. Read More

    Scleredema associated with Sjögren's syndrome.
    An Bras Dermatol 2015 May-Jun;90(3 Suppl 1):81-3
    Garcia de Orta Hospital, Almada, PT.
    Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Read More

    Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients.
    J Eur Acad Dermatol Venereol 2015 Dec 24;29(12):2399-404. Epub 2015 Aug 24.
    Service de Dermatopathologie, Paris, France.
    Background: The prognostic and therapeutic features of scleredema are poorly documented.

    Objectives: To describe the characteristics of patients with scleredema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions and course.

    Methods: We conducted a retrospective multicentre study. Read More

    Detection of renal brush border membrane enzymes for evaluation of renal injury in neonatal scleredema.
    Pak J Med Sci 2015 Jan-Feb;31(1):65-9
    Qiaozhi Yang, MD, Department of Pediatrics, Liaocheng People's Hospital, Liaocheng, Shandong252000, China.
    Objective: To evaluate renal brush border membrane enzymes in urine as an indicator for renal injury in neonatal scleredema (NS).

    Methods: Sixty nine NS patients in our hospital were enrolled and divided into mild group and moderate/severe group. Patients were further randomly divided into therapy and control subgroups for 7 days ligustrazine administration. Read More

    Clinical correlates of human leucocyte antigen (HLA)-G in systemic sclerosis.
    Clin Exp Immunol 2015 Jul 10;181(1):100-9. Epub 2015 May 10.
    Department of Biomedical Sciences and Human Oncology (DIMO), Rheumatologic and Systemic Autoimmune Diseases Unit, University of Bari Medical School, Bari, Italy.
    Human leucocyte antigen (HLA)-G has a tolerogenic function and could play a role in the pathogenesis of immune-mediated diseases, including systemic sclerosis (SSc). The aim of this study was to evaluate HLA-G serum expression (sHLA-G) and the HLA-G gene 14 base pairs (bp) insertion/deletion (del(-)/del(+)) polymorphism in patients with Ssc, to search for possible associations with clinical and laboratory variables. sHLA-G was measured by enzyme-linked immunosorbent assay (ELISA) in sera from 77 patients with SSc and 32 healthy donors (HD); the 14 bp del(-)/del(+) polymorphism was evaluated by polymerase chain reaction (PCR) amplification of peripheral blood mononuclear cells (PBMC) genomic DNA. Read More

    Scleredema adultorum of Buschke over an unusual site associated with pregnancy.
    Indian Dermatol Online J 2014 Oct;5(4):466-8
    Departments of Obstetrics and Gynaecology, MVJ Medical College and Research Hospital, Bangalore, Karnataka, India.
    Scleredema adultorum of Buschke is characterized by symmetrical, diffuse, non-pitting erythematous swelling of the skin due to accumulation of collagen and mucopolysaccharides in the dermis. Herein we report a case of scleredema over an unusual site in a pregnant woman. Read More


    [Scleredema adultorum Buschke and exulcerated necrobiosis lipoidica in diabetes mellitus type 1].
    Dtsch Med Wochenschr 2014 Oct 15;139(43):2184-7. Epub 2014 Oct 15.
    Klinikum für Hautkrankheiten, Universitätsklinikum Jena.
    History And Admission Findings: A 53-year-old woman suffering from brown plaques occasionally ulcerating at both lower legs and in the course of disease on the back of the foot presented at our department. Moreover she complained about an increasing sclerosis of her skin at the back over the last 15 years. She had suffered from diabetes mellitus type-1 for more than 40 years. Read More

    Scleredema--an uncommon cause of swelling in a child--a case report and review of the literature.
    BMC Res Notes 2014 Aug 27;7:571. Epub 2014 Aug 27.
    Department of Paediatrics, Nepalese Army Institute of Health Sciences, Shree Birendra Hospital, Swayambhu, Chhauni, Kathmandu, Nepal.
    Background: Body swelling in a child is a common symptom. Apart from systemic causes like renal, hepatic, and cardiac, rarely such a swelling may be caused by dermatologic conditions.

    Case Presentation: A child presented with swelling of the body which was subsequently diagnosed as scleredema, a rare and benign dermatologic condition. Read More

    Extensive scleredema adultorum with loss of eccrine glands.
    J Am Acad Dermatol 2014 Sep;71(3):e99-e101
    Department of Dermatology, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan; Institute of Biomedical Engineering, College of Medicine and College of Engineering, National Taiwan University, Taipei, Taiwan; Research Center for Developmental Biology and Regenerative Medicine, National Taiwan University, Taipei, Taiwan. Electronic address:

    Fulminans in dermatology: a call to action: a recommendation for consideration of the term scleredema fulminans.
    J Clin Aesthet Dermatol 2014 Jun;7(6):42-5
    Division of Dermatology, Cooper Medical School of Rowan University, Camden, New Jersey.
    The term fulminans is used infrequently in dermatology, being reserved for those cases of rapid onset with potentially severe sequelae or those that are life-threatening, thereby warranting urgent intervention. In this commentary, the authors propose that the term scleredema fulminans be utilized in severe, progressive cases of scleredema adultorum of Buschke presenting with rapid onset. Read More

    A case of scleredema adultorum successfully treated with narrow-band ultraviolet B phototherapy.
    Mod Rheumatol 2016 5;26(2):302-6. Epub 2014 Feb 5.
    a Department of Dermatology , University of Tokyo Graduate School of Medicine , Tokyo , Japan.
    Scleredema adultorum, also known as scleredema of Buschke, is a rare connective tissue disease with unknown etiology, which is characterized by diffuse skin induration of face, neck, upper chest, back, shoulders and arms. Although there is no established treatment for this disease, the efficacy of phototherapy has been reported. We herein describe a case of scleredema adultorum successfully treated with narrow-band ultraviolet B and discuss a potential mechanism explaining its efficacy for fibrotic skin diseases. Read More

    Morphological clues in the diagnosis of sclerodermiform dermatitis.
    Am J Dermatopathol 2014 Jun;36(6):449-64
    *Pathology Specialist, Department of Cellular Pathology, Hospital El Bierzo, Ponferrada, Spain; and Departments of †Resident (M.G-T.), Assistant Dermatologist (A.L.R-V.) Department of Dermatology, ‡Associate Professor, Pathology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, INER/CIENI, Hospital Angeles Lomas, Centro Medico ABC, Mexico City, México.
    In this report, we review 5 sclerodermiform cutaneous conditions: eosinophilic fasciitis, systemic nephrogenic fibrosis, scleredema, scleromyxedema, and toxic oil syndrome. We emphasize the morphological differences between the conditions and some morphological clues that are important to differential diagnosis. Read More

    Scleredema diabeticorum.
    Dermatol Online J 2013 Dec 16;19(12):20718. Epub 2013 Dec 16.
    New York University School of Medicine.
    We present a case of scleredema with a leonine facies in a 56-year-old man with a history of poorly controlled diabetes mellitus. The patient initially presented with erythematous, edematous papules and plaques on the face, neck, and upper back. Read More

    [Risk factors for prognosis of neonatal necrotizing enterocolitis: an analysis of 82 cases].
    Zhongguo Dang Dai Er Ke Za Zhi 2013 Dec;15(12):1082-5
    Department of Neonatology, Children's Hospital Affiliated to Soochow University, Suzhou, Jiangsu 215000, China.
    Objective: To study the risk factors for prognosis of neonatal necrotizing enterocolitis (NEC).

    Methods: A retrospective analysis was performed on the clinical data of 82 neonates with NEC confirmed between January 2008 and October 2012. The possible prognostic factors in NEC were investigated by logistic regression analysis. Read More

    Cutaneous indurated plaque on the abdomen associated with diabetes mellitus.
    Aust Fam Physician 2013 Dec;42(12):876-7
    MD, is a resident, Department of Dermatology, San Cecilio University Hospital, Granada, Spain.
    A woman, 74 years of age, presented to the emergency department with a lesion on the lower abdominal wall that had started a month earlier and was not associated with any other symptoms. Her family physician had treated it with emollient creams. Relevant past medical history included congestive heart failure, hypertension, hypertensive heart disease, pulmonary hypertension, mitral regurgitation, chronic atrial fibrillation, rosacea and diabetes mellitus that was being treated with oral hypoglycaemics (metformin). Read More

    Dermatologic manifestations of diabetes mellitus: a review.
    Endocrinol Metab Clin North Am 2013 Dec;42(4):869-98
    University of California, Irvine, Irvine, CA 92697, USA.
    Diabetes mellitus affects every organ of the body including the skin. Certain skin manifestations of diabetes are considered cutaneous markers of the disease, whereas others are nonspecific conditions that occur more frequently among individuals with diabetes compared with the general population. Diabetic patients have an increased susceptibility to some bacterial and fungal skin infections, which account, in part, for poor healing. Read More

    The differential diagnosis of systemic sclerosis.
    Curr Opin Rheumatol 2013 Nov;25(6):692-9
    aDepartment of Rheumatology bDepartment of Dermatology, University Hospital Basel, Basel, Switzerland.
    Purpose Of Review: The new American College of Rheumatology/European League Against Rheumatism classification criteria will enable earlier diagnosis and, therefore, the use of newer treatment modalities for systemic sclerosis (SSc). It is therefore critical to exclude non-SSc causes for diffuse skin thickening as early as possible.

    Recent Findings: The recently described gadolinium-induced nephrogenic systemic fibrosis may mimic SSc as may other conditions which require a different treatment strategy. Read More

    Localized cutaneous fibrosing disorders.
    Rheum Dis Clin North Am 2013 May 16;39(2):347-64. Epub 2013 Mar 16.
    Division of Immunology and Rheumatology, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94305, USA.
    This article acquaints the reader with disorders of the skin that might mimic systemic sclerosis but whose pathology is localized to the skin and/or has extracutaneous manifestations that are different than systemic sclerosis. These disorders include localized scleroderma (morphea), eosinophilic fasciitis, scleredema, scleromyxedema, nephrogenic systemic fibrosis, and chronic graft-versus-host disease. Particular emphasis is placed on clinical and histopathologic features that help the clinician differentiate between these disorders. Read More

    New side effect of TNF-alpha inhibitors: morphea.
    Skinmed 2013 Jan-Feb;11(1):59-60
    Department of Dermatology, University of Alabama School of Medicine, Birmingham, AL 35294-0009, USA.
    An otherwise healthy 45-year-old man with a 3-year history of poorly controlled psoriasis (no arthritis) was treated with etanercept 50-mg subcutaneous injections twice weekly for 3 months and then once weekly. Alternative treatment options were either unavailable (long commute for phototherapy) or contraindicated (history of alcohol abuse). The patient initially tolerated etanercept well with significant clinical improvement and had an uneventful course; however, approximately 18 months after initiating therapy, he abruptly developed dusky, indurated, and tender plaques on his abdomen and thighs at the sites of etanercept injections (Figure 1). Read More

    Scleredema in a Patient with AIDS-Related Lipodystrophy Syndrome.
    Case Rep Endocrinol 2013 10;2013:943798. Epub 2013 Jan 10.
    Division of Rheumatology, Cooper Medical School of Rowan University, Suite 262, E and R Building, The Haddon Avenue Office, Camden, NJ 08103, USA.
    Scleredema is a form of cutaneous mucinosis caused by an increased accumulation of collagen and mucin in the dermis. It is characterized by diffused, nonpitting swelling and induration of the skin. Scleredema diabeticorum is one type of scleredema associated with diabetes mellitus. Read More

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