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Melike Kalfa, Hayriye Koçanaoğulları, Figen Yargucu Zihni, Gonca Karabulut, Hakan Emmungil, Vedat İnal
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. The patient was followed regularly for 13 years and did not respond to various combinations of immunosuppressants and psoralen plus ultraviolet A therapy. Treatment of scleredema is quite difficult and of limited success. At present, there is no proved treatment for this disease...
December 2015: Eur J Rheumatol
Caroline M Lewis, April T Sanchez, Loretta S Davis
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.
August 30, 2016: Pediatric Dermatology
Yao An, Li Liu, Qiu-Yu Li, Ya-Lin Ran, Lu-Quan Li
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. RESULTS: The incidence rate of concurrent sepsis in NEC was 32.2% (88/273). The neonates with stage III NEC had a significantly higher incidence rate of concurrent sepsis than those with stage II NEC (69...
August 2016: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Luciana Rodino Lemes, Gabriele Medina Vilela, Sandra Maria Barbosa Durães, Enoi Aparecida Guedes Vilar
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. Several treatments are reported in the literature without well-established results...
May 2016: Revista da Associação Médica Brasileira
Zdeněk Adam, Petr Szturz, Marta Krejčí, Vladimír Vašků, Luděk Pour, Eva Michalková, Sabina Ševčíková, Zdeňka Čermáková, Karel Veselý, Jíří Vaníček, Eva Pourová, Zdeněk Král, Jiří Mayer
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). It is probably caused by the stimulation of mucin production in fibroblasts by immunoglobulins, frequently monoclonal immunoglobulin...
December 2015: Vnitr̆ní Lékar̆ství
Kaushik Ghosh, KapilDev Das, Susmita Ghosh, Sisir Chakraborty, Sanat Kumar Jatua, Ambarish Bhattacharya, Manas Ghosh
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...
September 2015: Indian Journal of Dermatology
Mariem Mohamed, Hichem Belhadjali, Amel Belhaj Béchir, Adnene Moussa, Jameleddine Zili
No abstract text is available yet for this article.
February 2016: International Journal of Dermatology
M N Shazzad, A K Azad, S J Abdal, R Afrose, M M Rahman, S A Haq
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. It may be classified into three clinical groups, each has a different history, course, and prognosis. Histology of skin is characterized by thickened dermis and increased spaces between large collagen bundles due to increased deposition of mucopolysaccharide in the dermis...
July 2015: Mymensingh Medical Journal: MMJ
João Alves, Tiago Judas, Tiago Ferreira, Diogo Matos, Elvira Bártolo
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. Further investigation led to the diagnosis of primary Sjögren's syndrome. The association between scleredema and autoimmune disorders has been rarely seen...
May 2015: Anais Brasileiros de Dermatologia
F Rongioletti, F Kaiser, E Cinotti, D Metze, M Battistella, P G Calzavara-Pinton, K Damevska, G Girolomoni, J André, J-L Perrot, W Kempf, B Cavelier-Balloy
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. RESULTS: We identified 44 patients (26 men).The mean age at diagnosis was 53.8 years. The most common associated disorders were endocrine/metabolic diseases including 30 patients suffering from diabetes, mostly type 2 diabetes...
December 2015: Journal of the European Academy of Dermatology and Venereology: JEADV
Soo-Eun Jung, You Chan Kim
No abstract text is available yet for this article.
August 2015: Annals of Dermatology
Qing Ren, Yongjun Zhang, Jinying Yang, Lixia Wei, Lili Zhao, Qiaozhi Yang
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. Urine samples were collected to detect renal brush border membrane enzymes (RBBME) by ELISA and β2-microglobulin (β2-MG) by radioimmunoassay (RIA)...
January 2015: Pakistan Journal of Medical Sciences Quarterly
E Favoino, I E Favia, S Vettori, C Vicenti, M Prete, G Valentini, F Perosa
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...
July 2015: Clinical and Experimental Immunology
Joana Castro Carvalho, Thaís Neves Costa, Heloisa Helena Gonçalves De Moura, Leonardo Pereira Quintella, Sueli Carneiro, Marcia Ramos-e-Silva
No abstract text is available yet for this article.
November 2014: Skinmed
C Sujatha Vinod, H Ambika, Hariharasubramony Ambika, Nithya Reddy, Jayantha Kumar De
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.
October 2014: Indian Dermatology Online Journal
A-K Dumke, J Tittelbach, P Elsner
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. INVESTIGATIONS: Skin biopsies form the ulcerated plaques showed changes typical for necrobiosis lipoidica...
October 2014: Deutsche Medizinische Wochenschrift
Asena Çiğdem Doğramacı, Mehmet Uğur Inan, Esin Atik, Cumali Gökçe
Scleredema is a rare connective tissue disorder that belongs to a group of scleroderma-like disorders. Although no known curative therapy exists, various specific treatments have been proposed in the literature. In this report, we describe five cases of scleredema partially treated with low-dose methotrexate therapy. All patients have diabetes mellitus type II. All patients were started on methotrexate 15 mg subcutaneously once weekly for 3 months. Biopsy specimens were taken from all patients and were examinated histologically before the treatment and after 3 months of treatment...
June 2012: Balkan Medical Journal
Bikash Shrestha, Arun Kumar Neopane, Rajesh Panth
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. Scleredema can be confused with similar sounding terms like scleroderma and scleromyxedema. CONCLUSIONS: The case is presented to highlight scleredema as a rare cause of body swelling in paediatrics and to differentiate it from similar sounding rare terms like scleroderma and scleromyxedema...
2014: BMC Research Notes
I-Chun Lin, Hsien-Yi Chiu, Jung-Yi Chan, Sung-Jan Lin
No abstract text is available yet for this article.
September 2014: Journal of the American Academy of Dermatology
Lacy L Sommer, Warren R Heymann
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.
June 2014: Journal of Clinical and Aesthetic Dermatology
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