Scleredema Research Articles

Electron beam dose perturbations caused by diode detectors used for in vivo dosimetry: Gafchromic film dose measurements in a realistic pelvic prosthesis phantom

Background and purposeDiode detectors used for in vivo dosimetry could influence patient dose due to their shadow effect in radiation fields. This study measures the impact of in vivo diodes on therapeutic electron beam dose distributions in a realistic pelvic prosthesis phantom. Materials and methodsTwo commercially available electron in vivo diodes (the IBA Dosimetry EDP-53G and Sun Nuclear QED 1112000) were studied. Depth dose measurements for 8 × 8, 10 × 10 and 11 × 11 cm2 15 MeV electron fields for open beams and with the diodes placed on the central axis (CAX) of the beam were acquired using Gafchromic EBT3 films in a realistic pelvic phantom that contains Ti hip prosthesis and bony structures. Deviations between dose data obtained with and without the diodes in the electron fields were used to evaluate the impact of the diodes on the electron dose inside the phantom. ResultsCAX depth dose distributions measured using Gafchromic EBT3 film with the diodes in the electron fields on the prosthesis and non-prosthesis sides of the phantom showed dose reductions in the shadow of the diodes compared to dose data acquired in open fields. The average dose reductions recorded along the CAX at depths ≤ 4.0 cm ranged from 6% to 8% and 10% to 13% for the QED and EDP diodes, respectively. In the treatment of epithelial skin cancer or scleredema of Buschke using 10 irradiation fractions, the diodes would reduce the electron dose by approximately 1% if only single IVD measurements are performed. For treatment regimes using 3–5 fractions a dose reduction ≥ 2% would be attained. ConclusionElectron in vivo diodes can induce strong perturbations on patient dose that could influence clinical outcome especially for single-fraction electron treatment of CD30+ lymphoproliferative disorders and accelerated partial breast irradiation delivered by intraoperative radiotherapy using a single dose after lumpectomy or a treatment regime using 3–5 fractions.

Scleredema adultorum of Buschke in a child confirmed by special stains

Scleredema adultorum of Buschke in a child confirmed by special stains

Ultrasonographic Findings of Scleredema Adultorum of Buschke Involving the Posterior Neck.

ObjectiveTo 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 MethodsAfter obtaining IRB approval, eight patients with scleredema adultorum of Buschke were enrolled. They underwent US examination of their posterior neck. The diagnoses were confirmed pathologically. The clinical history and US images were evaluated retrospectively. Dermal thickness was compared between the patient group and the age- and sex-matched control group.ResultsThe patients included seven males and one female with a mean age of 51.5 years. All patients presented with thickening of the skin and/or a palpable mass on the posterior neck. Five (62.5%) of the eight patients showed erythematous discoloration. Six patients (75.0%) had a history of diabetes. The Hemoglobin A1c level was found to be increased in all patients. US images did not show any evidence of a soft tissue mass or infection. The mean dermal thickness in patients (7.01 ± 1.95 mm) was significantly greater than that in the control group (3.08 ± 0.87 mm) (p = 0.001). Multiple strong echogenic spots in the dermis were seen in all patients. Seven patients (87.5%) showed posterior shadowing in the lower dermis.ConclusionWhen a patient with a history of diabetes presents with a palpable mass or erythematous discoloration of the posterior neck and US shows the following imaging features: 1) no evidence of a soft tissue mass or infection, 2) thickening of the dermis, 3) multiple strong echogenic spots and/or posterior shadowing in the dermis, scleredema adultorum of Buschke should be considered in the differential diagnosis.

Pathophysiological Mechanisms in Sclerosing Skin Diseases.

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. A better understanding of the mechanisms underlying these events is prerequisite for developing novel targeted therapeutic approaches.

Cutaneous Manifestations of Diabetes Mellitus: A Review.

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. Noninfectious as well as infectious diseases have been described as dermatologic manifestations of diabetes mellitus. Moreover, diabetic neuropathy and angiopathy may also affect the skin. Pruritus, necrobiosis lipoidica, scleredema adultorum of Buschke, and granuloma annulare are examples of frequent noninfectious skin diseases. Bacterial and fungal skin infections are more frequent in people with diabetes. Diabetic neuropathy and angiopathy are responsible for diabetic foot syndrome and diabetic dermopathy. Furthermore, antidiabetic therapies may provoke dermatologic adverse events. Treatment with insulin may evoke local reactions like lipohypertrophy, lipoatrophy and both instant and delayed type allergy. Erythema multiforme, leukocytoclastic vasculitis, drug eruptions, and photosensitivity have been described as adverse reactions to oral antidiabetics. The identification of lesions may be crucial for the first diagnosis and for proper therapy of diabetes.

Diabetes mellitus and congenital proximal femur hypoplasia: is there a link or association

We present a case of congenital short femur with scleredema Diabeticorum (SD) in a patient with diabetic neuropathy with unusual skin lesion distribution. Gait symptoms progressively worsened. The atypical distribution of the skin lesions is quite unusual. This case express rare complications of diabetes mellitus, the maternal diabetes which had disrupted in part the development of skeletal system of the offspring who in turn developed a rare dermatologic complications i.e. scleredema Diabeticorum (SD).This case will leave inedible memory for every diabetologist not to miss the skeletal and dermatologic manifestations of DM. The combination of this congenital proximal femoral hypoplasia with complicating diabetes make the question if there is a link with similar genetic predilection or just an association.

Comparison of cutaneous manifestations of diabetic with nondiabetic patients: A case-control study

Background: Diabetes mellitus is a metabolic disorder characterized by raised fasting and postprandial blood glucose levels and a variety of multisystem complications. The prevalence of skin manifestations seems to be similar between type 1 and type 2 diabetes mellitus patients. Cutaneous manifestations usually develop following the diagnosis of diabetes, but in some patients, they are the initial presenting signs, thereby helping in the early diagnosis of diabetes mellitus. Objectives: The objective of this study is to describe the cutaneous lesions in patients with type 2 diabetes mellitus and to compare the pattern of skin manifestations in diabetics and nondiabetics in coastal Karnataka and neighboring districts of Kerala. Methodology: This case-control study included 400 patients with type 2 diabetes mellitus and cutaneous manifestations attending the outpatient Departments of Dermatology and General Medicine and those admitted under these departments. A total of 400 age and sex-matched nondiabetic patients were included as controls. Results: Cutaneous infections were noted in 148 (37%) diabetics of which fungal infections were the most common seen in 106 (26.5%) patients, followed by xerosis in 121 (30.25%) and acrochordons in 71 (17.75%) patients. Other dermatoses associated with diabetes mellitus noted were acanthosis nigricans (5.5%), scleredema diabeticorum (0.25%), diabetic bullae (0.5%), and Kyrle's disease (1%). Cutaneous changes associated with neurovascular complications included diabetic foot in 3%, diabetic dermopathy in 2%, and pigmented purpuric dermatosis in 0.25% of the diabetics. Conclusion: A joint effort between dermatology and general medicine is necessary for the early recognition and treatment of the skin conditions and also to ensure adequate metabolic control.

CLINICAL CASE – SCLEREDEMA ADULTORUM

Scleredema of Buschke is a rare dermal disease of unknown etiology which is characterized by a dense diffuse swelling of the derm and subcutaneous adipose tissue. It characterized by induration of the skin which includes a non-pitting hardening of the skin around the neck, shoulders, and trunk sometimes the face. Three basic forms are distinguished: classical, idiopathic, diabetic or metabolic. The histopathological features of scleredema are characterized by sharply thickened derma, edema and structural impairment of collagenous fascicles metachromasia appears in the foci of the damage as a result of accumulation of glycosaminoglycans. Differential diagnostics was carried out at the other diseases similar to scleredema such as systemic scleroderma, eosinophilic fasciitis, porphyria cutanea tarda hereditaria, porphyria cutanea scleroderma-like form, Werner's syndrome, scleromyxedema, amyloidosis. Several treatments are suggested in the literature. The present case record describes scleredema adultorum Buschke in a 52- years-old male with stiffness and density of the skin of trunk. He had suffered from diabetes mellitus type-1 for more than 30 years. The patient was treated with antibiotics, corticosteroids, polyenzyme agent, hepatoprotector, cytoflavin, polyvitamins. There was improvement with slightly decrease infiltration in foci lesions, skin color in the area of rash brightened. Skin diseases are common in diabetes mellitus. This clinical case will enable to expand knowledge of general practitioners about the rare dermal disease – Buschke's scleredema giving the possibility to reveal such patients at early stages of the disease in proper time.

Idiopathic scleredema

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.We present a case of scleredema in a 52-year-oldman with no identifiable associated condition,who experienced improvemement with UVBphototherapy.

Effect observation of gastrointestinal nutrition therapy on patients with neonatal scle-redema and nursing experience

Objective To study effect of gastrointestinal nutrition therapy on patients with neonatal scleredema and nursing methods. Methods A total of 70 patients with neonatal scleredema were selected and divided into control group(31 cases)and observation group(39 cases). The control group adopted the routine nursing, based on the control group gastrointestinal nutrition therapy was used in the observation group. Treatment time and treatment effect were compared between groups. Results In the observation group patients'body temperature recovery time, scleredema disappearing time, the length of hospitalization time were significantly shorter than those in the control group. Healing and efficient were significantly higher than those in the control group. The differences were statistically significant with comparison between the two groups(P<0.05). Conclusions The implementation of gastrointestinal nutrition therapy on patients with neonatal scleredema is safe and effective and has important significance in clinic. Key words: Neonatal scleredema; Gastrointestinal nutrition therapy; Nursing; Effect

Scleredema of Buschke Following Streptococcal Infection.

Dear Editor: Scleredema is characterized by non-pitting hardening of the skin that typically begins on the neck and subsequently spreads to the shoulders, back, and face. Histopathologically, it is characterized by excessive mucin deposition between collagen bundles. It is clinically classified into 3 types:postinfectious (type I), paraproteinemia-associated (type II), and diabeticorum (type III)1,2,3,4. Here, we report a case of of following a streptococcal infection. An 8-year-old boy presented with a 1-week history of skin firmness of the whole body. He experienced a febrile upper-respiratory infection 2 months prior with abrupt onset of skin hardening starting from his posterior neck and rapidly extending to his face, trunk, arms, and legs. On physical examination, whole-body and non-pitting induration of the skin were noted, especially on the posterior neck and upper back (Fig. 1). A biopsy from the posterior neck revealed dermal thickening with a square-like appearance, and a high-magnification view revealed slight widening of collagen bundles with normal fibroblast number (Fig. 2A, B). Mucin deposition was detected between collagen bundles (Fig. 2C). No other laboratory abnormalities except an elevated antistreptolysin O titer (476 U/L [normal range: 0~160 U/L]) were noted. Therefore, he was diagnosed with of following a streptococcal infection. Fig. 1 Whole-body and non-pitting induration of the skin was noted including face. On physical examination, it was hard to pinch his check. Fig. 2 (A) Skin biopsy specimen revealed a non-tapered appearance on a low-power field (H&E, ×40). (B) Fibroblasts were normal in number, and the spaces between collagen bundles were widened (H&E, ×400). (C) Mucin deposits were ... Scleredema is also termed of Buschke, adultorum, or of Buschke. The term adultorum was suggested by in 1902 to distinguish this entity from sclerema neonatorum2,3. It is usually classified into 3 types; however, the nomenclature of each subgroup has not been established. Moreover, classification systems differ among sources. Type I, known as the classic type, is characterized by a sudden onset of skin hardening after an acute febrile illness in children and adolescents. The preceding infection is usually bacterial or viral in origin, most commonly a streptococcal upper-respiratory infection. Thus, we suggest referring to this type postinfectious scleredema,1 to distinguish it from other types of or restricting the use of the term of Buschke to describe type I as it was first described by himself. Furthermore, the term adultorum should be avoided because it may cause confusion among physicians. Meanwhile, type II has an insidious onset and is usually associated with paraproteinemia, hypergammaglobulinemia, or multiple myeloma. Finally, type III scleredema, also called diabeticorum, is related to poorly controlled type 2 diabetes. However, there are other cases associated with insulinoma, secondary hyperparathyroidism, rheumatoid arthritis, or HIV infection that do not meet the criteria of any of the above mentioned type4. Type I is usually resolved within 2 years and does not require treatment. Thus, our patient has not been treated and has shown a resolution of symptoms over the past 5 months. If symptoms persist, phototherapy including psoralen plus ultraviolet A, ultraviolet A-1, and narrow-band ultraviolet B as well as radiotherapy and intravenous immunoglobulins should be considered. In conclusion, a classification system for must be established. In addition, type I should be named postinfectious scleredema or of Buschke.

The influence of sunflower oil daub on the incidence of nosocomial infection in hospitalized premature

Objective To discuss whether sunflower oil daub will have effect in treating premature or not.Methods 194 cases of premature whose weight were less than 2 kg in our newborns emergency center from December 2011 to April 2014 were randomly divided into experimental group and control group.In the premise of same primary care,experimental group received sunflower oil daub within 72 hours after birth,while control group did not receive any skin care products.Compared the incidence of sepsis,skin infections and mortality of two groups.Results There were 12 cases of infection in experimental group,without hypothermia,iatrogenic skin damage; there were 37 cases of infection,7 cases of hypothermia,15 cases of iatrogenic skin damage in control group; with statistically significant differences between two groups (P ＜ 0.01).Conclusions Sunflower oil daub could improve premature's skin barrier function,reduce exogenous bacteria invaded through the skin,reduce incidence of infection,hypothermia and iatrogenic skin damage.In experimental group,the incidence of scleredema descended,weight rapidly gain,hospitalization time was shortened. Key words: Premature; Sunflower oil daub; Complication

Improvement in Clinical Symptoms of Scleredema Diabeticorum by Frequency-Modulated Electromagnetic Neural Stimulation: A Case Report

Scleredema diabeticorum is a rare complication of diabetes, characterized by diffuse induration of the skin over the neck, shoulders, and upper trunk and arms, progressively leading to limited upper body motility and impaired respiratory function (1). Etiology is unknown, and histopathological findings include thickened dermis with abundant mucin deposition among large collagen bundles. No treatment is currently available. We report a case of scleredema diabeticorum treated with frequency-modulated electromagnetic neural stimulation (FREMS), a transcutaneous electrotherapy that proved effective and safe in the treatment of symptomatic diabetic neuropathy (2). Putative mechanisms of FREMS include enhancement of microvascular blood flow (3) and vasomotor activity mediated by smooth cells (4) and release of vascular endothelial growth factor. In April 2012, a 55-year-old man with type 1 diabetes since age 11 presented complaining of a progressive thickening and hardening of his skin, deterioration in daily life activities …

Scleredema diabeticorum in a nonregulated type 2 diabetic patient

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Scleredema adultorum of Buschke in a child – a rare case

Scleredema adultorum of Buschke is a rare connective tissue disorder reported usually following streptococcal infection, influenza, measles, and mumps. This is a report of scleredema adultorum developing after streptococcal infection in an 10-year-old girl. The diagnosis was established by characteristic picture on skin biopsy using special stain. The patient had a benign course and responded to local emollients only. Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-3, 60-62 DOI: http://dx.doi.org/10.3126/jcmsn.v9i3.10225

UVA-1 therapy of localized scleroderma and other diseases accompanied by skin sclerosis

The study results confirm the efficacy of the UVA-1 therapy for patients with localized scleroderma, extragenital lichen sclerosus et atrophicus, sclerodermatous chronic graft-versus-host disease and scleredema adultorum. The therapy has an anti-inflammatory and anti^m^ action, arrests progression and reduces the activity of the disease process, and has a good efficacy and safety profile.

Clinical analysis of eight cases of scleredema

Objective To assess the clinical characteristics of patients with scleredema.Methods This retrospective study included eight patients with scleredema hospitalized in the Peking Union Medical College Hospital (PUMCH) from 1998 to 2012.Data were collected from medical records.Clinical manifestations,pathological findings and therapeutic outcomes were analyzed.Results There were four male patients and four female patients with an average age of 44 years.Sclerotic induration of the skin on the neck,shoulders and back were observed in 6 (75％) patients,and multi-systemic involvement in 6 patients.Three of five patients with type 2 diabetes millitus (DM) suffered from diabetes-associated complications.Lanbda type M-protein was observed in one patient,and Kappa type M-protein in one patient.Two patients also suffered from fever,and one from Sj?gren's syndrome.Pathological examination of biopsy specimens from four patients revealed thickening of the whole dermis as well as marked hyperpasia of collagen.Alcian blue staining was positive in the space between collagen fibers.The condition was improved in three patients treated with glucocorticoid and immunosuppressants as well as in one patient receiving benzylpenicillin treatment,but poorly responded to ultraviolet therapy in one patient.Conclusions It is recommended to monitor blood glucose and serum immunoglobulins as well as to screen for the involvement of multiple systems and occurrence of multiple myeloma,lymphoma and other malignancies among patients with scleredema.

Successful treatment of scleredema diabeticorum by combining local psoralen plus ultraviolet A light phototherapy and colchicine

Successful treatment of scleredema diabeticorum by combining local psoralen plus ultraviolet A light phototherapy and colchicine

Scleredema in a Patient with AIDS-Related Lipodystrophy Syndrome

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. AIDS-related insulin resistance and lipodystrophy syndrome are a newly emerging entities in HIV-infected patients associated with severe metabolic disturbances and insulin resistance. The long-standing diabetes in these patients may contribute to the development of scleredema diabeticorum. Here, we report the rare occurrence of scleredema in an HIV-infected patient with AIDS-related lipodystrophy syndrome.

Scleredema Diabeticorum Partially Treated with Low-Dose Methotrexate: a Report of Five Cases

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. All cases partially responded to low-dose methotrexate therapy. We believe that methotrexate therapy may be an alternative therapeutic options in scleredema in view of its efficacy.