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Int J Dermatol 2020 Jun 12. Epub 2020 Jun 12.

Dermatology Unit, Hospital Universitario San Cecilio, Granada, Spain.

Clin Dermatol 2020 Mar - Apr;38(2):235-249. Epub 2019 Oct 24.

Department of Rheumatology and Immunology, University of Pécs Clinical Center, Pecs, Hungary. Electronic address:

Sclerodermalike syndromes (SLSs) comprise diseases with mucin deposition (eg, scleromyxedema, scleredema), with eosinophilia (eg, eosinophilic fasciitis), metabolic or biochemical abnormalities (eg, nephrogenic systemic fibrosis), or endocrine disorders (eg, POEMS syndrome, or polyneuropathy, organomegaly, endocrinopathy, monoclonal lymphoproliferative disorder, and hypothyroidism). Chronic graft-versus-host disease may also show sclerodermalike skin changes. Inherited progeria syndromes with early aging (eg, Werner syndrome) and a heterogeneous group of hereditary disorders with either skin thickening (eg, stiff skin syndrome) or atrophy and tightening (eg, acrogeria) can also imitate classic systemic sclerosis (SSc). Read More

J Eur Acad Dermatol Venereol 2020 Mar 12;34(3):e133-e135. Epub 2019 Dec 12.

Department of Dermatology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea.

JAAD Case Rep 2019 Nov 24;5(11):940-942. Epub 2019 Oct 24.

Department of Dermatology, University of Rochester Medical Center, Rochester, New York.

Australas J Dermatol 2020 Feb 10;61(1):e110-e111. Epub 2019 Sep 10.

Department of Dermatology, Waikato Hospital, Hamilton, New Zealand.

Panminerva Med 2019 Jul 26. Epub 2019 Jul 26.

Department of Pediatric, The 960th Hospital of PLA, Jinan, China -

Internist (Berl) 2019 Aug;60(8):783-791

Hautklinik, Universitätsklinikum Erlangen, Ulmenweg 18, 91054, Erlangen, Deutschland.

Sclerosing skin manifestations are more a symptom than a diagnosis and must therefore be meticulously clarified. Systemic scleroderma as a multiorgan disease must be distinguished from localized scleroderma or morphea because in addition to a different clinical picture they have a different prognosis and necessitate other therapeutic procedures. Rare sclerosing skin diseases with implications for internal medicine are eosinophilic fasciitis, Buschke's scleredema adultorum, scleromyxedema and nephrogenic systemic fibrosis. Read More

Acta Med Port 2019 Jun;32(6):459-465

Clínica Universitária de Dermatologia. Hospital de Santa Maria. Lisbon. Portugal.

Diabetes is a serious, chronic disease with a rising prevalence worldwide. Its complications are a major cause of morbidity and mortality and contribute substantially to health care costs. In this article the authors review the most common and sensitive skin manifestations that can be present on patients with diabetes and prediabetes. Read More

J Cardiothorac Surg 2019 Jun 26;14(1):118. Epub 2019 Jun 26.

Departments of Medical Oncology, Linyi Central Hospital, No.17 Jiankang Road, Yishui County, Linyi, 276400, Shandong Province, China.

Background: An increasing number of hospitals have carried out neonatal thoracoscopic assisted repair of congenital diaphragmatic hernia (CDH).

Methods: The 26 cases received thoracoscopic-assisted repair (observation group) and 44 cases open repair (control group). General anesthesia was performed with endotracheal intubation using a trachea cannula without cuff. Read More

Ann Nutr Metab 2019 16;74(4):339-347. Epub 2019 May 16.

Department of Pediatrics, Novosibirsk Medical University, Novosibirsk, Russian Federation.

Background: Youth-onset type 2 diabetes (T2D) is increasing in many countries, creating large personal and societal burdens. While many primary health-care professionals (HCPs) are aware of the classic symptoms of T2D, there are several other manifestations that could indicate its presence.

Summary: This narrative review summarizes information on these symptoms and indicators, focusing on those less well known. Read More

J Med Case Rep 2019 May 14;13(1):145. Epub 2019 May 14.

National Hospital of Sri Lanka, Colombo, Sri Lanka.

Background: Scleredema is a rare sclerodermoid skin condition characterized by diffuse symmetrical thickening of the upper part of the body. Its association with monoclonal gammopathy and myeloma was recently described; very few cases have been reported to date.

Case Presentation: A 66-year-old Sri Lankan woman who had been followed in a dermatology unit for 34 years with diffuse systemic sclerosis presented with an acute exacerbation of the skin disease. Read More

Acta Dermatovenerol Croat 2019 Mar;27(1):16-21

Amer Ali Almohssen, MD , Dermatopathology Fellow, State University of New York, Downstate Medical School & The Ackerman Academy of Dermatopathology, New York City , New York, USA;

Reticular erythematous mucinosis (REM) is a rare form of primary cutaneous mucinosis, most often involving the midline of the upper chest or back in middle-aged women. REM bears clinical and histopathologic resemblance to lupus erythematosus tumidus (LET), dermatomyositis, scleredema, and lichen myxedematosus. Early recognition and diagnosis of REM is particularly relevant to exclude the abovementioned diseases, as REM is more benign and has fewer systemic consequences. Read More

Zhongguo Gu Shang 2019 Jan;32(1):85-87

Department of Hand Surgery, Ningbo No.6 Hospital, Ningbo 315000, Zhejiang, China;

Dermatol Ther 2019 05 4;32(3):e12854. Epub 2019 Mar 4.

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

Intravenous immunoglobulins (IVIG) are therapeutic products, comprising polyclonal IgGs, which are obtained from human plasma pool of healthy blood donors. Despite the lack of Food and Drug Administration (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) 2020 May 1;154(9):371-372. 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 2020 May-Jun;86(3):272-277

"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 2019 Mar 28;33(3):e104-e106. Epub 2019 Feb 28.

Department of Immuno-Hematology, Saint-Louis Hospital, Assistance Publique - Hôpitaux de Paris, Hôpital Saint-Louis, 1 avenue Claude Vellefaux, 75010, Paris, France.

Z Rheumatol 2019 Feb;78(1):14-23

Klinik für Rheumatologie und klinische Immunologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.

Background: Systemic sclerosis (SSc) is characterized by heterogeneous clinical symptoms. Peripheral skin fibrosis can be a common symptom. Nevertheless, a variety of diseases with different etiologies are associated with a thickening of the skin and make the initial diagnosis of systemic sclerosis more difficult. 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 2019 ;15(2):172-175

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

J Dtsch Dermatol Ges 2018 May 30;16(5):624-626. Epub 2018 Mar 30.

Universitätshautklinik, Eberhard-Karls-Universität Tübingen, Germany.

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 02 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

Hautarzt 2017 Nov;68(11):929-931

Klinik für Dermatologie, Dermatochirurgie und Allergologie, Asklepios Klinik Weißenfels, Naumburger Str. 76, 06667, Weißenfels, Deutschland.

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