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

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

Dermatol Ther 2017 Jun 2. 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

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

Int J Dermatol 2017 May 2. Epub 2017 May 2.

Istituto Dermopatico dell'Immacolata-IRCCS, FLMM, Rome, Italy.

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

Eur J Rheumatol 2016 Dec 1;3(4):191-192. Epub 2016 Feb 1.

Department of Internal Medicine, Division of Rheumatology, Oakwood Hospital, Dearborn, Michigan, USA.

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

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

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

JAMA 2016 Aug;316(5):543

Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, Ohio.

JAMA 2016 Aug;316(5):542

Spectrum Health Regional Laboratory, Grand Rapids, Michigan.

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

Cutis 2016 May;97(5):348;351;352

Departments of Dermatology and Pathology, Cleveland Clinic, Ohio, USA.

JAMA 2016 Mar;315(11):1159-60

Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, Ohio.

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

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

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

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

Int J Dermatol 2016 Feb 4;55(2):e100-2. Epub 2015 Nov 4.

Department of Dermatology, Fattouma Bourguiba University Hospital, Monastir, Tunisia.

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

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

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

Ann Dermatol 2015 Aug 29;27(4):478-80. Epub 2015 Jul 29.

Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.

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

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

Skinmed 2014 Nov-Dec;12(6):337-40

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

Diabetes Care 2014 Nov;37(11):e233-4

Diabetes Research Institute, San Raffaele Scientific Institute and Vita-Salute San Raffaele University, Milan, Italy

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

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

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:

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

JAMA Dermatol 2014 Jul;150(7):788-9

Department of Dermatology, Brigham and Women's Hospital, Boston, Massachusetts3Harvard Medical School, Boston, Massachusetts.

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

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

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

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

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

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

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

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

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

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

Methods Mol Biol 2013 ;961:287-303

Centre LOEX de L'Université Laval, Génie tissulaire et régénération, LOEX, Québec, QC, Canada.

Skin fibrosis is involved in several pathologies as hypertrophic scar or scleroderma. The determination of the mechanisms at the origin of these problems is however difficult due to the low number of in vivo models. To bypass this absence of animal models, studies typically use human pathological cells cultured in a monolayer way on plastic. Read More

Case Rep Dermatol 2012 Sep 27;4(3):265-8. Epub 2012 Sep 27.

Dermatology Unit, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

Scleredema is a rare condition of unknown pathogenesis. It is classically divided into three types, based on its association with postinfection (usually streptococcal), monoclonal gammopathy and diabetes mellitus (scleredema diabeticorum). Scleredema diabeticorum often has a subtle onset, persistent involvement and is refractory to therapies. Read More

J Dermatol 2013 Jan 18;40(1):77-8. Epub 2012 Oct 18.

Arch Dermatol 2012 Oct;148(10):1126-8

Divisions of Immunology, Allergy, and Infectious Diseases, Medical University of Vienna, Vienna, Austria.

Leuk Lymphoma 2013 Jun 15;54(6):1324-6. Epub 2012 Nov 15.

BMJ Case Rep 2012 Oct 6;2012. Epub 2012 Oct 6.

Department of Pediatrics, Memorial University, St. John's, Canada.

Scleredema adultorum is a rare condition characterised by progressive collagen and mucin deposition in the skin. While the aetiology has not been clearly delineated, the condition is often associated with common infections. The current report describes a previously healthy 16-year-old boy who presented with 3 weeks of progressive neck swelling and skin induration. Read More