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Dermatol Ther 2019 Feb 13:e12854. Epub 2019 Feb 13.

Department of Dermatology, Yildirim Beyazit University, Medical School, Ankara, Turkey.

Intravenous immunoglobulins are therapeutic products, comprising polyclonal IgGs, which are obtained from human plasma pool of healthy blood donors. Despite the lack of FDA approval, the experience of using IVIG in various dermatological diseases increases day by day and exciting results are reported. However, experience with the use of IVIG in dermatological indications are mostly case reports whereas randomized, controlled, double-blind, multicentric studies have not been performed. Read More

Med Clin (Barc) 2019 Feb 1. Epub 2019 Feb 1.

Unidad de Diabetes, Endocrinología y Nutrición, Hospital General de Granollers, Granollers, Barcelona, España.

PLoS One 2018 31;13(12):e0210023. Epub 2018 Dec 31.

Department of Biomedical Sciences and Human Oncology (DIMO), Rheumatologic and Systemic Autoimmune Diseases Unit, University of Bari Medical School, Bari, Italy.

Carbamylation is a post-translational modification that mostly affects proteins with low turnover, such as dermal proteins. Carbamylated proteins accumulate in skin in an age-dependent manner, contributing to tissue alterations. As dermis is affected by systemic sclerosis (SSc) and anti-carbamylated protein antibodies (anti-CarP Ab) are found in SSc patients, we sought to evaluate the specificity of anti-CarP Ab and their relationship with clinical parameters reflecting skin involvement in SSc. Read More

Zhonghua Wei Zhong Bing Ji Jiu Yi Xue 2018 Nov;30(11):1046-1050

Department of Pediatrics, the First Affiliated Hospital of Bengbu Medical College, Bengbu 233004, Anhui, China (Chen X, Peng WS, Zhang Z, Zhao Q, Zhou Y, Chen L); Department of Pediatric, Anhui Provincial Hospital, the First Affiliated Hospital of University of Science and Technology of China, Hefei 230001, Anhui, China (Pan JH). Corresponding author: Pan Jiahua, Email:

Objective: To evaluate the efficacy and safety of selective brain hypothermia (SBH) in the treatment of neonates with moderate or severe neonatal hypoxic-ischemic encephalopathy (HIE), and the effect of SBH treatment on serum levels of neuron-specific enolase (NSE) and central nervous specific protein S100.

Methods: A prospective randomized controlled trial was conducted. From January 2015 to June 2017, 42 children with moderate to severe HIE in the neonatal intensive care unit (NICU) of the First Affiliated Hospital of Bengbu Medical College were enrolled, and they were randomly divided into SBH treatment group and routine treatment group after obtaining the consent of the guardian of the children. Read More

Indian J Dermatol Venereol Leprol 2018 Oct 4. Epub 2018 Oct 4.

"Mario Aresu" Department of Medical Science, Section of Dermatology, University of Cagliari, Cagliari, Italy.

Scleredema adultorum of Buschke is a rare fibromucinous, scleroderma-like connective tissue disease most commonly found in a post-infectious setting or linked to hematological disorders or diabetes. Lichen sclerosus et atrophicus is an autoimmune condition only in 2.5% of cases localized exclusively at an extragenital site, occurring in up to 34% of patients in association with other autoimmune diseases such as vitiligo, thyroid disorders, alopecia areata, lichen planus, morphea, pernicious anemia and systemic lupus erythematosus. Read More

J Eur Acad Dermatol Venereol 2018 Sep 29. Epub 2018 Sep 29.

Hôpitaux de Paris, Saint-Louis Hospital, Department of Immuno-hematology, Paris, France.

The presence of a serum monoclonal immunoglobulin can lead to complications related to the physiochemical properties of circulating immunoglobulin and/or its deposition in different tissues, such as kidney, peripheral nervous system and skin. Specific cutaneous manifestations are rare. They are referred to as a Monoclonal Gammopathy of Cutaneous Significance (MGCS). Read More

Rev Med Interne 2018 Nov 22;39(11):891-893. Epub 2018 Sep 22.

Service de médecine interne, hôpital Saint-Éloi, 80, boulevard Augustin-Fliche, 34295 Montpellier, France.

Hautarzt 2018 Nov;69(11):908-915

Klinik für Dermatologie, Venerologie und Allergologie, HELIOS St. Elisabeth Klinik Oberhausen, Universität Witten/Herdecke, Josefstr. 3, 46045, Oberhausen, Deutschland.

Scleredema adultorum (SA) is a rare scleromucinous disease of unknown etiology that characteristically leads to wodden induration located on the neck and upper trunk. Three types of SA can be distinguished according to the association with pre-existing or underlying disease: SA in association with infections (mostly streptococcal infections of the upper respiratory tract), SA in association with monoclonal gammopathy, and SA in association with diabetes. The clinical findings, extent of disease, and course substantially differ depending on the subtype of SA. Read More

Curr Rheumatol Rev 2018 08 7. Epub 2018 Aug 7.

Department of Clinical Pathology, Faculty of Medicine, Ain Shams University, El-Abaseya. Egypt.

Background: Scleroderma or systemic sclerosis (SSc) is a rare systemic autoimmune disease. Many conditions mimic the presentation of SSc, especially skin thickening and fibrosis. One of these conditions is scleredema, a rare collagen and mucin deposition disorder which was found to be associated with diabetes mellitus, streptococcal infection or monoclonal gammopathy. Read More

Case Rep Endocrinol 2018 16;2018:9513768. Epub 2018 Apr 16.

Interfaith Medical Center, Brooklyn, NY, USA.

Scleredema diabeticorum is a rare cutaneous manifestation of diabetes mellitus. We present a case of an obese male with poorly controlled diabetes who came to the hospital with upper back pain and subsequently developed sepsis due to a small deep-seated abscess in his back that was drained and treated with antibiotics. He was also found to have extensive induration of the skin over his back and neck. Read More

Korean J Radiol 2018 May-Jun;19(3):425-430. Epub 2018 Apr 6.

Department of Pathology, Dong-A University College of Medicine, Busan 49201, Korea.

Objective: To describe the clinical and ultrasonographic (US) findings in patients with scleredema adultorum of Buschke, who presented with sclerotic skin on their posterior neck.

Materials And Methods: After obtaining IRB approval, eight patients with scleredema adultorum of Buschke were enrolled. They underwent US examination of their posterior neck. Read More

Rev Med Interne 2018 May 12;39(5):360-363. Epub 2018 Mar 12.

Service de médecine interne, hôpital Victor-Provo, 11, boulevard Lacordaire, 59170 Roubaix, France.

Background: Scleredema adultorum (Buschke's scleredema) is a cutaneous mucinosis of unknown origin, clinically characterized by a diffuse induration of the skin usually involving the neck, shoulders and back, which limits patients' mobility.

Case Report: We report a case of a 50-year-old woman who presented a chronic sclerodermiform syndrome for 2 years associated with type 1 diabetes. Physical examination revealed an extensive skin induration involving the shoulders, neck and back. Read More

Diabetes Care 2018 Apr 26;41(4):e40-e41. Epub 2018 Jan 26.

Department of Dermatology, The First Affiliated Hospital, Nanjing Medical University, Nanjing, Jiangsu, China

G Ital Dermatol Venereol 2018 Apr 24;153(2):208-215. Epub 2018 Jan 24.

Department of Molecular Medicine, "Sapienza" University, Rome, Italy.

Scleroderma is divided into a systemic form called systemic sclerosis and a localized form also called morphea. According to 2013 ACR/EULAR Classification Criteria for Systemic Sclerosis, developed by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR), skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for a patient to be classified as having scleroderma. Histological examination is not included in the diagnostic criteria and is not routinely performed. Read More

Georgian Med News 2017 Dec(273):41-46

Shupyk National Medical Academy of Postgraduate Education; Ukrainian Research and Practical Centre of Endocrine Surgery, Transplantation of Endocrine Organs and Tissues of the Ministry of Health of Ukraine.

Skin pathology is registered in vast majority of patients with diabetes mellitus (DM). Despite the abundance of publications on dermatological problems in DM, there is still a number of gaps to be discussed in terms of pathophysiological mechanisms. The goal of this review was to assess the mechanisms of development of different skin pathologies under DM. Read More

J Eur Acad Dermatol Venereol 2018 Feb 23;32(2):e84-e85. Epub 2018 Jan 23.

NHS Greater Glasgow & Clyde, Glasgow, UK.

Clin Dermatol 2018 Jan - Feb;36(1):94-100. Epub 2017 Sep 8.

Department of Dermatology, Istanbul Medeniyet University, Medical School, Istanbul, Turkey. Electronic address:

The link between the metabolic syndrome (MetS) and skin diseases is increasingly important, with new associations being discovered. The association between MetS and psoriasis or MetS and hidradenitis suppurativa is well known, although the relationship between MetS and various autoimmune or inflammatory diseases has only recently attracted interest. Some inflammatory skin diseases, such as vitiligo, scleredema, recurrent aphthous stomatitis, Behçet disease, rosacea, necrobiosis lipoidica, granuloma annulare, skin tags, knuckle pads, and eruptive xanthomas, have possible associations with MetS. Read More

Acta Derm Venereol 2018 Mar;98(3):305-309

Department of Dermatology, Jena University Hospital Erfurter Straße 35, DE-07743 Jena, Germany.

Scleroedema adultorum Buschke is a rare skin disease, which can be divided into 3 subtypes: classic type, occurring after respiratory infections; a type lacking association with infections; and a type associated with diabetes. Scleroedema adultorum Buschke is characterized by thickening and tightening of the skin, which typically starts at the neck. In half of patients, spontaneous remission may occur. Read More

Australas J Dermatol 2018 Aug 25;59(3):e215-e217. Epub 2017 Sep 25.

Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University, Yonago, Japan.

Scand J Rheumatol 2018 07 12;47(4):342-343. Epub 2017 Sep 12.

a Department of Pediatrics, Allergy and Immunology Unit, Advanced Pediatrics Centre , Postgraduate Institute of Medical Education and Research , Chandigarh , India.

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

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

Disabil Rehabil 2018 11 24;40(23):2833-2835. 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

Clin Rev Allergy Immunol 2017 Dec;53(3):306-336

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 2018 Apr;14(1):22-27

Fortis Escorts Hospital, Jaipur, Rajasthan, India.

Background: 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.

Objective: 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 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

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 10 2;56(10):e193-e194. 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

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

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