118 results on '"Scleredema Adultorum diagnosis"'
Search Results
2. [Differential diagnosis of sclerosing skin diseases: scleredema adultorum of Buschke].
- Author
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Pfeiffer C and Sunderkötter C
- Subjects
- Humans, Diagnosis, Differential, Skin pathology, Immunoglobulins, Intravenous therapeutic use, Scleredema Adultorum diagnosis, Skin Diseases diagnosis
- Abstract
Scleredema adultorum of Buschke is an important differential diagnosis in sclerosing diseases. Diagnosis is based on the typical histology with mucin deposits and grossly increased dermal width and a clinical presentation of diffuse non-pitting induration of the skin starting at the nape of the neck and interscapular region extending to shoulders and upper thorax, causing dysmobility due to dermal stiffness. Even though the pathogenesis remains unclear, three subtypes can be distinguished: association with infections, paraproteins, or most frequently with diabetes mellitus. Management of the disease includes physiotherapy, physical therapies such as ultraviolet (UV) or ionizing irradiation, intravenous immunoglobulins and interdisciplinary treatment directed at associated diseases. Optimizing diabetes therapy and thereby decreasing insulin use may confer significant improvement., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)
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- 2024
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3. Indurated plaques on the back: distinguishing stiff skin syndrome from scleredema and morphea.
- Author
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Raef HS, Nassim JS, and Fedeles F
- Subjects
- Humans, Skin, Contracture, Scleredema Adultorum diagnosis, Scleroderma, Localized complications, Scleroderma, Localized diagnosis, Skin Diseases, Genetic diagnosis
- Published
- 2022
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4. Scleredema adultorum of Buschke associated with hypothyroidism and liver cirrhosis.
- Author
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Muralidharan S, Arun K, Ruckmani V, and Devi B
- Subjects
- Humans, Male, Middle Aged, Alopecia etiology, Betamethasone therapeutic use, Fusidic Acid therapeutic use, Glucocorticoids therapeutic use, Pruritus etiology, Thyrotoxicosis complications, Hypothyroidism complications, Liver Cirrhosis complications, Scleredema Adultorum diagnosis
- Abstract
Scleredema adultorum of Buschke is a rare disorder of the connective tissue, involving the skin. Here, we present a 61-year-old male, who is a known case of compensated liver cirrhosis with a past history of being treated for autoimmune thyrotoxicosis, who presented with complaints of alopecia, skin tightening, dry skin, pruritus, and woody indurated plaques on the skin of the upper back, shoulder, and arms. Skin biopsy of the arm revealed the characteristic features of scleredema. He was extensively evaluated for known literature-cited causes of scleredema, and the work up revealed a negative result. He was also found to be hypothyroid on presentation. Hence, we present a case of scleredema occurring in a patient with hypothyroidism and chronic liver disease, which to our knowledge is being described for the first time in literature., Competing Interests: None
- Published
- 2021
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5. Screening for the presence of scleroedema adultorum of Buschke in patients with diabetes mellitus: newly diagnosed patients had a high prevalence of dyslipidaemia.
- Author
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Csonka V, Bódis B, Kovács D, Farkas N, Kálmán E, Czirják L, and Varjú C
- Subjects
- Dyslipidemias etiology, Female, Humans, Hydroxymethylglutaryl-CoA Reductase Inhibitors therapeutic use, Lipids blood, Logistic Models, Male, Mass Screening, Middle Aged, Prevalence, Risk Factors, Scleredema Adultorum etiology, Scleredema Adultorum pathology, Skin pathology, Diabetes Mellitus, Type 2 complications, Dyslipidemias epidemiology, Scleredema Adultorum diagnosis
- Abstract
Background: Scleroedema adultorum of Buschke is a rare disorder characterized by fibromucinous thickening of the dermis that manifests mainly at the nape of the neck and on the upper back and shoulders. This study screened patients with diabetes mellitus for skin hardening caused by scleroedema adultorum of Buschke and characterized the clinical and laboratory findings in patients with newly identified cases, with a focus on lipid metabolism abnormalities and vascular complications., Methods: Out of 113 consecutive patients with diabetes, 11 (9.7%) new scleroedema patients, all with type 2 diabetes, were found. Their clinical and laboratory data were compared to those of the rest of the screened patients and to those of a cohort of 15 patients with scleroedema and diabetes who were already being treated in a tertiary clinical centre at the University of Pécs., Results: Higher proportions of patients with dyslipidaemia, hypertriglyceridemia (P < 0.05) and increased mean levels of non-high-density lipoprotein cholesterol (non-HDL-C) were found (P < 0.01) in both scleroedema groups than in the group without scleroedema. Stroke and venous thromboembolism (VTE) were more frequently found in the histories of both the newly identified scleroedema group (each 3/11; 27.3%) and the treated cohort (each 6/15; 40.0%) than in the group without scleroedema (6/102; 5.9% in cases of stroke P = 0.021, P < 0.001; and 14/102; 13.7%; P < 0.05 in cases of VTE, respectively). Based on binary logistic regression, a high non-HDL-C level (odds ratio (OD): 3.338, confidence interval (CI): 1.77-6.28; P < 0.001) and insulin treatment (OR 7.64, CI 1.9-29.3; P = 0.003) were independent predictors of scleroedema in patients with diabetes mellitus., Conclusions: Diabetes patients with scleroedema had more severe dyslipidaemia and higher occurrence of vascular complications compared to those without scleroedema. In addition to poorly controlled type 2 diabetes mellitus requiring insulin treatment, high non-HDL-C levels may be another contributing factor to the development of scleroedema., Trial Registration: NCT04335396 .
- Published
- 2021
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6. Dermoscopic-pathological correlations in a case of granuloma gluteale adultorum.
- Author
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Payapvipapong K and Tanaka M
- Subjects
- Granuloma diagnosis, Humans, Scleredema Adultorum diagnosis, Scleredema Adultorum pathology
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- 2020
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7. Woody Erythematous Induration on the Posterior Neck.
- Author
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Faraj Y, Gray T, Chavda K, Gibbons G, and Miller R
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- Adult, Female, Humans, Neck, Scleredema Adultorum pathology, Diabetes Mellitus, Type 1 complications, Scleredema Adultorum diagnosis
- Published
- 2020
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8. Scleredema diabeticorum: Description of 11 cases.
- Author
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Simó-Guerrero O, Recasens-Gracia A, and Giménez-Pérez G
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- Humans, Skin, Scleredema Adultorum diagnosis
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- 2020
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9. Scleredema of Buschke associated with lichen sclerosus: Three cases.
- Author
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Venturi M, Damevska K, Ferreli C, Pinna AL, Atzori L, Gocev G, and Rongioletti F
- Subjects
- Aged, Female, Humans, Middle Aged, Lichen Sclerosus et Atrophicus complications, Lichen Sclerosus et Atrophicus diagnosis, Scleredema Adultorum complications, Scleredema Adultorum diagnosis
- Abstract
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. In particular, a stronger link with an autoimmune background in lichen sclerosus et atrophicus has been observed in women who showed higher prevalence for autoimmune conditions and circulating autoantibodies. Literature reveals a genetic susceptibility linked to specific HLA types. We report three patients who developed lichen sclerosus et atrophicus superimposed on skin involved by scleredema adultorum of Buschke. Although the association of lichen sclerosus et atrophicus with scleredema adultorum of Buschke could be coincidental, both diseases could be considered part of the spectrum of sclerodermoid disorders with common underlying pathogenetic mechanisms; which could explain the sequential or simultaneous occurrence of both lesions in our patients., Competing Interests: None
- Published
- 2020
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10. [Mimetics of systemic sclerosis].
- Author
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Jendrek ST, Kahle B, and Riemekasten G
- Subjects
- Diagnosis, Differential, Eosinophilia diagnosis, Fasciitis diagnosis, Humans, Scleredema Adultorum diagnosis, Scleromyxedema diagnosis, Skin, Synovitis, Connective Tissue Diseases diagnosis, Scleroderma, Localized diagnosis, Scleroderma, Systemic diagnosis
- Abstract
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., Objective: The different disease entities that can lead to dermal fibrosis should be differentiated. An earlier diagnosis of SSc would therefore be facilitated., Methods: A literature search was carried out for clinical pictures that can be associated with skin fibrosis. The clinical picture, the etiology and the treatment of the individual diseases are described., Results: Diseases that can mimic the cutaneous symptoms of SSc include morphea, scleroderma, diabetic cheirarthritis, scleromyxedema, nephrogenic systemic fibrosis and eosinophilic fasciitis. The characteristic pronounced skin involvement, an accompanying Raynaud's phenomenon, capillary microscopy, histopathology and antinuclear antibodies help to enable a differentiation of SSc from its mimics., Conclusion: An early differential diagnostic distinction between SSc and other sclerosing diseases is important due to SSc-associated and potentially life-threatening systemic organ involvement. If a diagnosis of SSc has been made, a critical and organ-specific evaluation with respect to pulmonary, gastrointestinal, renal and cardiac involvement is mandatory and should be repeated at regular intervals.
- Published
- 2019
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11. An Unusual Presentation of a Rare Scleroderma Mimic: What is Behind the Scenes?
- Author
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Ibrahim RA, Abdalla NEH, Shabaan EAH, and Mostafa NBH
- Subjects
- Diagnosis, Differential, Female, Humans, Middle Aged, Multiple Myeloma diagnosis, Scleroderma, Systemic diagnosis, Scleroderma, Systemic pathology, Multiple Myeloma complications, Scleredema Adultorum diagnosis, Scleredema Adultorum etiology, Scleredema Adultorum pathology
- Abstract
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., Case Presentation: A 55 years old female presented with insidious onset and progressive course of diffuse skin thickening of face, neck, arms, forearms, thighs, chest, back, and excluding both hands and feet of 6 years' duration associated with arthralgia, dysphagia and dyspnea on exertion of 1- year duration. There was no history of Raynaud's phenomenon. Erythrocyte sedimentation rate was 100 mm/1st h, autoantibodies for SSc were negative, nail fold capillaroscopy normal, pulmonary function tests showed restrictive pattern and high-resolution computed tomography showed interstitial lung fibrosis. Patient was not fulfilling the American collage of rheumatology/European League Against Rheumatism classification criteria for SSc. Skin biopsy was done and revealed histological appearance of scleredema. Investigations were done for disease association with scleredema. The patient was not diabetic, antistreptolysin O titer was normal, serum protein electrophoresis, immunofixation and bone marrow biopsy were done, and the patient was diagnosed as scleredema associated with immunoglobulin A kappa multiple myeloma. Treatment by combination of bortezomib, cyclophosphamide, and dexamethasone was started with marked clinical and hematological improvement., Conclusion: Many conditions mimic SSc including scleredema, which may be the initial presentation of multiple myeloma. Rheumatologists and dermatologists should be able to recognize these conditions to provide the suitable management and follow-up for these patients., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)
- Published
- 2019
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12. [Diffuse pain in a 63-year-old man].
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Bélénotti P, Moureau JP, Vacheret H, Piro J, Vivarrat-Perrin L, Benyamine A, and Guilpain P
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- Chronic Pain etiology, Diagnosis, Differential, Humans, Male, Middle Aged, Scleredema Adultorum complications, Chronic Pain diagnosis, Scleredema Adultorum diagnosis
- Published
- 2018
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13. [Scleredema adultorum : Clinical presentation, diagnostic workup, differential diagnosis, treatment option].
- Author
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Kreuter A
- Subjects
- Diagnosis, Differential, Humans, Methotrexate, Neck, Phototherapy, Scleredema Adultorum diagnosis
- Abstract
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. Spontaneous regression often occurs in infection-associated SA, whereas patients with diabetes or monoclonal gammopathy usually show a chronic progressive course of disease. Phototherapy and methotrexate are the current recommended first-line treatments for SA, clinical improvement often takes several months, and treatment failure is frequent. Physiotherapy should be offered in all types of SA in order to improve motility.
- Published
- 2018
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14. Serositis in a child with scleredema of Buschke: an unusual association and improvement following glucocorticoids.
- Author
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Sharma D, Somasekhara-Aradhya A, Vignesh P, Saikia UN, and Singh S
- Subjects
- Child, Preschool, Facies, Female, Humans, Pleural Effusion complications, Radiography, Thoracic, Respiratory Tract Infections, Scleredema Adultorum complications, Scleredema Adultorum pathology, Serositis complications, Glucocorticoids therapeutic use, Pleural Effusion diagnostic imaging, Scleredema Adultorum diagnosis, Serositis diagnostic imaging, Skin pathology
- Published
- 2018
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15. Ultrasonographic Findings of Scleredema Adultorum of Buschke Involving the Posterior Neck.
- Author
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Ha DH, Lee MJ, and Kim SJ
- Subjects
- Adult, Female, Glycated Hemoglobin analysis, Humans, Male, Middle Aged, Neck pathology, Retrospective Studies, Scleredema Adultorum diagnostic imaging, Scleredema Adultorum pathology, Skin physiopathology, Ultrasonography, Neck diagnostic imaging, Scleredema Adultorum diagnosis
- Abstract
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. 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., Results: The 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., Conclusion: When 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.
- Published
- 2018
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16. Treatment of Scleroedema Adultorum Buschke: A Systematic Review.
- Author
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Miguel D, Schliemann S, and Elsner P
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- Dermatologic Agents adverse effects, Humans, Immunosuppressive Agents adverse effects, PUVA Therapy, Photopheresis, Remission, Spontaneous, Risk Factors, Scleredema Adultorum diagnosis, Scleredema Adultorum etiology, Scleredema Adultorum radiotherapy, Skin pathology, Skin radiation effects, Treatment Outcome, Dermatologic Agents therapeutic use, Immunosuppressive Agents therapeutic use, Scleredema Adultorum drug therapy, Skin drug effects
- Abstract
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. The aim of this systematic review is to summarize all reported treatments for scleroedema adultorum Buschke, based on articles from PubMed database, using the query "scleroedema adultorum Buschke treatment", English and German, published between 1970 and 2016 and documenting adequate treatments. The results are based mainly on individual case reports, small case series, and retrospective studies often reporting unsuccessful results. Treatment options include topical as well as systemic treatments, and physical modalities. There is a need for randomized controlled trials and studies on long-term outcomes after treatment.
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- 2018
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17. [Painless, soft, livid erythematous swelling on the neck and upper part of the back].
- Author
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Kohn D, Ziemer M, Meier T, and Wetzig T
- Subjects
- Biopsy, Diabetes Complications pathology, Diabetes Mellitus, Type 1 diagnosis, Diabetes Mellitus, Type 1 pathology, Diagnosis, Differential, Edema pathology, Erythema pathology, Humans, Male, Middle Aged, Risk Factors, Scleredema Adultorum pathology, Skin pathology, Back pathology, Diabetes Complications diagnosis, Edema etiology, Erythema etiology, Neck pathology, Scleredema Adultorum diagnosis
- Published
- 2017
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18. Scleredema of Buschke with prominent periorbital edema.
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Fania L, Colonna L, Pagnanelli G, Abeni D, Annessi G, and Mazzanti C
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- Eyelids, Humans, Male, Middle Aged, Scleredema Adultorum complications, Edema etiology, Scleredema Adultorum diagnosis, Scleredema Adultorum pathology
- Published
- 2017
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19. European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis.
- Author
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Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, Cutolo M, Rongioletti F, Denton CP, Rudnicka L, Frasin LA, Smith V, Gabrielli A, Aberer E, Bagot M, Bali G, Bouaziz J, Braae Olesen A, Foeldvari I, Frances C, Jalili A, Just U, Kähäri V, Kárpáti S, Kofoed K, Krasowska D, Olszewska M, Orteu C, Panelius J, Parodi A, Petit A, Quaglino P, Ranki A, Sanchez Schmidt JM, Seneschal J, Skrok A, Sticherling M, Sunderkötter C, Taieb A, Tanew A, Wolf P, Worm M, Wutte NJ, and Krieg T
- Subjects
- Diagnosis, Differential, Humans, Nephrogenic Fibrosing Dermopathy pathology, Scleredema Adultorum pathology, Scleromyxedema pathology, Nephrogenic Fibrosing Dermopathy diagnosis, Nephrogenic Fibrosing Dermopathy therapy, Scleredema Adultorum diagnosis, Scleredema Adultorum therapy, Scleromyxedema diagnosis, Scleromyxedema therapy
- Abstract
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. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this guideline provides clinicians with an overview of the diagnosis and treatment of scleromyxedema, scleredema (of Buschke) and nephrogenic systemic sclerosis (nephrogenic fibrosing dermopathy)., (© 2017 European Academy of Dermatology and Venereology.)
- Published
- 2017
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20. Idiopathic scleredema.
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Ng E, Rosenstein R, Terushkin V, Meehan S, and Pomeranz MK
- Subjects
- Back, Humans, Male, Middle Aged, Scleredema Adultorum pathology, Scleredema Adultorum diagnosis
- Abstract
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.
- Published
- 2016
21. Laboratory Testing in the Setting of Diabetic Scleredema--Reply.
- Author
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Chatterjee S
- Subjects
- Humans, Male, Diabetes Mellitus, Type 2 complications, Scleredema Adultorum diagnosis, Scleredema Adultorum etiology
- Published
- 2016
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22. Laboratory Testing in the Setting of Diabetic Scleredema.
- Author
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Alter D
- Subjects
- Humans, Male, Diabetes Mellitus, Type 2 complications, Scleredema Adultorum diagnosis, Scleredema Adultorum etiology
- Published
- 2016
- Full Text
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23. Benign lesion on the posterior aspect of the neck.
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Brankov N, Moore B, Messana K, and Piliang M
- Subjects
- Diagnosis, Differential, Humans, Male, Middle Aged, Collagen metabolism, Fibroma diagnosis, Fibroma metabolism, Fibroma pathology, Head and Neck Neoplasms diagnosis, Head and Neck Neoplasms metabolism, Head and Neck Neoplasms pathology, Lipoma diagnosis, Scleredema Adultorum diagnosis, Skin metabolism, Skin pathology
- Published
- 2016
24. Neck Pain, Stiffness, and Periorbital Edema in a Man With Diabetes.
- Author
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Chatterjee S
- Subjects
- Edema etiology, Headache etiology, Humans, Male, Middle Aged, Neck pathology, Neck Pain etiology, Scleredema Adultorum pathology, Diabetes Mellitus, Type 2 complications, Scleredema Adultorum diagnosis, Scleredema Adultorum etiology
- Published
- 2016
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25. [Monoclonal immunoglobulin (M-Ig) and skin diseases from the group of mucinoses--scleredema adultorum Buschke and scleromyxedema. Description of four cases and an overview of therapies].
- Author
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Adam Z, Szturz P, Krejčí M, Vašků V, Pour L, Michalková E, Ševčíková S, Čermáková Z, Veselý K, Vaníček J, Pourová E, Král Z, and Mayer J
- Subjects
- Aged, Female, Humans, Immunologic Factors therapeutic use, Male, Middle Aged, Scleredema Adultorum diagnosis, Scleredema Adultorum therapy, Scleromyxedema diagnosis, Scleromyxedema therapy, Immunoglobulin G immunology, Immunoglobulins, Intravenous therapeutic use, Scleredema Adultorum immunology, Scleromyxedema immunology
- Abstract
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. Therefore these diseases are typically associated with monoclonal gammopathy., Case Reports: We describe a cohort of 4 patients, skin manifestations were twice identified as scleredema and twice as scleromyxedema. All the four patients had type IgG monoclonal immunoglobulin and had clonal plasma cells in the bone marrow proven by histologic examination and flow cytometry. Therefore we commenced chemotherapy in all of them. In one case this chemotherapy was ended by a high-dose chemotherapy with transplanting of autologous red blood cells. This therapy attained the complete disappearance of monoclonal immunoglobulin as well as cutaneous and extracutaneous manifestations of scleredema (obstipation). In one case chemotherapy led to partial hematologic remission and partial improvement of skin manifestations. The other two patients did not respond to standard chemotherapy. The condition of one of them resulted in dermato-neuro syndrome (confusion, somnolence passing into coma and grand mal seizure) and improved following an intensive treatment including also intravenous application of immunoglobulins in a dose of 2 g/per 1 kg weight. This patient has now been under long-term treatment with these immunoglobulins, during which the skin symptoms have significantly diminished, but the concentration of monoclonal immunoglobulin has not changed. The fourth patient not responding to standard chemotherapy was treated with intravenous immunoglobulins also in a dose of 2 g/per 1 kg of weight 1× in a month. After 4 applications the thickening of skin and subcutaneous tissue moderately diminished, so the range of possible movement of the upper limbs and neck became larger and the itchy skin morphs which accompanied the disease disappeared completely., Conclusion: It is possible to use chemotherapy and high-dose chemotherapy in the treatment of mucinosis associated with monoclonal gammopathy, as in the treatment of multiple myeloma. If such treatment is not possible or it has not attained disappearance of monoclonal immunoglobulin, improvement can be achieved through repeated application of intravenous immunoglobulins. The treatment with intravenous immunoglobulins in an immunomodulation dose of 2 g/per 1 kg of weight effects the moderation of skin manifestations, but it does not lead to the decrease in monoclonal immunoglobulin.
- Published
- 2015
26. Scleredema Diabeticorum - A Case Report.
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Shazzad MN, Azad AK, Abdal SJ, Afrose R, Rahman MM, and Haq SA
- Subjects
- Combined Modality Therapy, Diagnosis, Differential, Humans, Male, Middle Aged, PUVA Therapy, Scleredema Adultorum therapy, Dermatologic Agents administration & dosage, Diabetes Mellitus, Type 2, Methotrexate administration & dosage, Scleredema Adultorum diagnosis
- Abstract
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. Differential diagnosis can be made considering the typical clinical features and the histological peculiarity. No therapy is effective. In this communication we have presented a 54 year old man with scleredema successfully treated by PUVA and methotrexate. We reviewed associated diseases, clinical and histopathological characteristics, evolution and response to treatment.
- Published
- 2015
27. [Scleredema adultorum Buschke and exulcerated necrobiosis lipoidica in diabetes mellitus type 1].
- Author
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Dumke AK, Tittelbach J, and Elsner P
- Subjects
- Biopsy, Combined Modality Therapy, Diabetes Mellitus, Type 1 pathology, Diagnosis, Differential, Disease Progression, Female, Humans, Leg Dermatoses diagnosis, Leg Dermatoses pathology, Leg Dermatoses therapy, Middle Aged, Necrobiosis Lipoidica pathology, Scleredema Adultorum pathology, Skin pathology, Diabetes Mellitus, Type 1 diagnosis, Necrobiosis Lipoidica diagnosis, Scleredema Adultorum diagnosis
- Abstract
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. Biopsies of the back were diagnosed as scleredema adultorum Buschke. Laboratory tests displayed a normal antinuclear antibody titer. The differential diagnosis of systemic sclerosis could be ruled out., Treatment and Course: We repeatedly performed debridement of ulcers, cream-PUVA therapy, applied a vacuum-sealing and topical corticosteroids at the lower legs. Meanwhile she received oral pentoxyfillin. The scleredema was treated with UVA-1 phototherapy but had to be stopped for high photosensitivity. Additionally physiotherapy was prescribed. Nonetheless the course of disease was chronic and therapy-resistant., Conclusion: Skin diseases are common in diabetes mellitus. Necrobiosis lipoidica and scleredema adultorum Buschke are rare complications but often refractory to treatment., (© Georg Thieme Verlag KG Stuttgart · New York.)
- Published
- 2014
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28. Cutaneous indurated plaque on the abdomen associated with diabetes mellitus.
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Salazar-Nievas M, Crespo-Lora V, Rubio-López J, and Arias-Santiago S
- Subjects
- Aged, Diagnosis, Differential, Female, Humans, Diabetes Complications diagnosis, Scleredema Adultorum diagnosis
- Abstract
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). Physical examination revealed an area of skin on the lower abdominal wall that was ill-defined and indurated, with whitish papules and a 'cobblestone' appearance (Figure 1).
- Published
- 2013
29. The differential diagnosis of systemic sclerosis.
- Author
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Tyndall A and Fistarol S
- Subjects
- Connective Tissue Diseases diagnosis, Diagnosis, Differential, Eosinophilia, Humans, Nephrogenic Fibrosing Dermopathy diagnosis, Scleredema Adultorum diagnosis, Scleroderma, Localized diagnosis, Scleromyxedema diagnosis, Synovitis diagnosis, Scleroderma, Systemic diagnosis
- Abstract
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. Recently, treatment with immunoablation and autologous stem cell transplantation has been shown to significantly benefit some patients with conditions such as scleromyxoedema and SSc. The more accurate measurement of SSc-specific autoantibodies such as topoisomerase 1, centromere and RNA polymerase has recently allowed a more precise subclassification of SSc with implications for treatment and prognosis., Summary: Skin thickening is a nonspecific manifestation of many different processes including (rarely) early scleroderma, which is mostly symmetrical and associated with Raynaud's phenomenon, nailfold capillaroscopic changes and antinuclear antibodies. If the latter three factors are absent, then other conditions must be excluded, the commonest being eosinophilic fasciitis. Skin biopsy (looking for eosinophil infiltration, increased mucin or amyloid deposition), SSc-specific autoantibodies or paraproteins in blood and a careful medical history and system screening will exclude nonscleroderma conditions.
- Published
- 2013
- Full Text
- View/download PDF
30. Complete remission of multiple myeloma associated scleredema after bortezomib-based treatment.
- Author
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Szturz P, Adam Z, Vašků V, Feit J, Krejčí M, Pour L, Hájek R, and Mayer J
- Subjects
- Antineoplastic Agents therapeutic use, Boronic Acids therapeutic use, Bortezomib, Humans, Male, Middle Aged, Multiple Myeloma drug therapy, Phenotype, Pyrazines therapeutic use, Remission Induction, Scleredema Adultorum diagnosis, Multiple Myeloma complications, Scleredema Adultorum etiology
- Published
- 2013
- Full Text
- View/download PDF
31. Localized cutaneous fibrosing disorders.
- Author
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Yaqub A, Chung L, Rieger KE, and Fiorentino DF
- Subjects
- Chronic Disease, Diagnosis, Differential, Eosinophilia diagnosis, Eosinophilia physiopathology, Fasciitis diagnosis, Fasciitis physiopathology, Graft vs Host Disease diagnosis, Graft vs Host Disease physiopathology, Humans, Interdisciplinary Communication, Nephrogenic Fibrosing Dermopathy diagnosis, Nephrogenic Fibrosing Dermopathy physiopathology, Scleredema Adultorum diagnosis, Scleredema Adultorum physiopathology, Scleroderma, Localized diagnosis, Scleroderma, Localized physiopathology, Scleroderma, Systemic physiopathology, Scleromyxedema diagnosis, Scleromyxedema physiopathology, Skin Diseases physiopathology, Skin Diseases therapy, Scleroderma, Systemic diagnosis, Skin Diseases diagnosis
- Abstract
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. Treatment options are briefly reviewed., (Copyright © 2013 Elsevier Inc. All rights reserved.)
- Published
- 2013
- Full Text
- View/download PDF
32. Incomplete POEMS syndrome presenting for years solely with scleredema and hyperpigmentation.
- Author
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Ono S, Otsuka A, Miyachi Y, and Kabashima K
- Subjects
- Diagnosis, Differential, Female, Humans, Middle Aged, Time Factors, Hyperpigmentation diagnosis, POEMS Syndrome diagnosis, Scleredema Adultorum diagnosis
- Published
- 2013
- Full Text
- View/download PDF
33. Scleredema adultorum of Buschke: with unusual manifestations in a young female.
- Author
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Mohanasundaram K, Kumarasamy S, Kumar R, and Rajendran CP
- Subjects
- Adolescent, Edema etiology, Female, Humans, Larynx pathology, Esophageal Motility Disorders etiology, Hoarseness etiology, Scleredema Adultorum complications, Scleredema Adultorum diagnosis
- Published
- 2012
- Full Text
- View/download PDF
34. Scleroderma mimics.
- Author
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Nashel J and Steen V
- Subjects
- Diagnosis, Differential, Eosinophilia etiology, Eosinophilia therapy, Fasciitis etiology, Fasciitis therapy, Humans, Nephrogenic Fibrosing Dermopathy etiology, Nephrogenic Fibrosing Dermopathy therapy, Scleredema Adultorum etiology, Scleredema Adultorum therapy, Scleroderma, Localized etiology, Scleroderma, Localized therapy, Scleromyxedema etiology, Scleromyxedema therapy, Eosinophilia diagnosis, Fasciitis diagnosis, Nephrogenic Fibrosing Dermopathy diagnosis, Scleredema Adultorum diagnosis, Scleroderma, Localized diagnosis, Scleromyxedema diagnosis
- Abstract
Scleroderma is a rare systemic autoimmune disease with multiple organ manifestations, including skin fibrosis. The groups of disorders classified as scleroderma mimics share the common thread of skin thickening but are otherwise quite incongruous in terms of underlying disease process and other organ involvement. This article reviews the clinical presentation, etiology, and treatment options available for scleroderma mimics, including morphea, scleredema, diabetic cheiroarthropathy, scleromyxedema, nephrogenic systemic fibrosis, and eosinophilic fasciitis. Through greater understanding of these diseases and the associated extradermal implications, we hope to facilitate recognition of scleroderma and its mimics.
- Published
- 2012
- Full Text
- View/download PDF
35. Juvenile scleredema of Buschke.
- Author
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Rani JD, Patil SG, Murthy ST, Koshy AV, Nagpal D, and Gupta S
- Subjects
- Anti-Bacterial Agents therapeutic use, Child, Elasticity, Facial Muscles pathology, Female, Follow-Up Studies, Humans, Muscular Diseases diagnosis, Skin Physiological Phenomena, Facial Dermatoses diagnosis, Scleredema Adultorum diagnosis
- Abstract
Aim: To recognize a line of treatment for scleredema of Buschke in an adolescent., Background: Scleredema of Buschke is an uncommon disorder 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 variants are recognized. The histopathologic features of scleredema are characterized by thickened collagen bundles within the reticular dermis that are separated by mucin containing fenestrations. No consistent treatment modality is currently followed., Case Report: The present case report describes scleredema of Buschke in a 10-year-old female child reported with stiffness of facial skin and difficulty in opening the mouth. The patient was treated with antibiotics and vitamin supplements and there was drastic improvement with decrease in skin stiffness and increase in mouth opening. The stiffness of facial muscles decreased and mouth opening increased., Conclusion: Multiple treatment modalities for scleredema have been used. In the present case, high-dose antibiotic therapy was used with good prognosis., Clinical Significance: High-dose antibiotic therapy can be used for treatment of dermatologic disorders with reasonably good prognosis.
- Published
- 2012
- Full Text
- View/download PDF
36. Asymmetric childhood scleredema.
- Author
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Galan-Gutierrez M, Ruiz-Villaverde R, Márquez-Lobo B, Martinez De Victoria JM, and Sierra-Córcoles C
- Subjects
- Anti-Inflammatory Agents, Non-Steroidal therapeutic use, Child, Female, Humans, Indomethacin therapeutic use, Physical Therapy Modalities, Scleredema Adultorum pathology, Scleredema Adultorum therapy, Scleredema Adultorum diagnosis
- Published
- 2012
- Full Text
- View/download PDF
37. Scleromyxedema vs scleredema: a diagnostic challenge.
- Author
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Conde Fernandes I, Sanches M, Velho G, Lobo I, Alves R, and Selores M
- Subjects
- Adult, Diagnosis, Differential, Disease Progression, Humans, Immunoglobulins, Intravenous administration & dosage, Immunologic Factors administration & dosage, Male, Scleromyxedema drug therapy, Scleredema Adultorum diagnosis, Scleromyxedema diagnosis
- Published
- 2011
- Full Text
- View/download PDF
38. Case of diabetic scleredema: diagnostic value of magnetic resonance imaging.
- Author
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Kurihara Y, Kokuba H, and Furue M
- Subjects
- Collagen metabolism, Diabetes Complications metabolism, Diabetes Mellitus, Type 2 complications, Glycosaminoglycans metabolism, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Scleredema Adultorum metabolism, Skin metabolism, Skin pathology, Subcutaneous Tissue metabolism, Subcutaneous Tissue pathology, Diabetes Complications diagnosis, Scleredema Adultorum diagnosis
- Abstract
We report the case of a patient with a 20-year history of diabetes mellitus type 2 who developed sclerotic skin lesions on his neck and upper back. Physical and histological findings were compatible with diabetic scleredema. T(2)-weighted magnetic resonance imaging (MRI) revealed diffuse thickening of the dermis and subcutaneous tissue, with hyperintense signals. The MRI findings in the dermis corresponded with the accumulation of collagen tissue with deposition of glycosaminoglycans., (© 2011 Japanese Dermatological Association.)
- Published
- 2011
- Full Text
- View/download PDF
39. Scleredema of Buschke in a child with cardiac involvement.
- Author
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Isaac A, Costa I, and Leal I
- Subjects
- Captopril therapeutic use, Child, Collagen analysis, Digoxin therapeutic use, Furosemide therapeutic use, Heart Failure drug therapy, Heart Failure etiology, Humans, Magnetic Resonance Imaging, Male, Mucins analysis, Scleredema Adultorum complications, Skin chemistry, Water Deprivation, Heart Failure diagnosis, Scleredema Adultorum diagnosis
- Abstract
A few descriptions of scleredema of Bushke associated with cardiac involvement in children have been reported. We report a case of an 11-year-old boy with diffuse nonpitting edema of the trunk and face associated with cardiac involvement.
- Published
- 2010
- Full Text
- View/download PDF
40. [Clinical and pathologic characteristics of patients with scleredema].
- Author
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Du W, Sun QN, Yuan X, and Fang K
- Subjects
- Adolescent, Adult, Aged, Child, Child, Preschool, Female, Humans, Male, Neck pathology, Retrospective Studies, Scleredema Adultorum complications, Scleredema Adultorum diagnosis, Young Adult, Immunoglobulins blood, Scleredema Adultorum pathology, Skin pathology
- Abstract
Objective: To investigate the clinical and pathologic characteristics of patients with scleredema., Method: The clinical and pathologic data of 67 outpatients with scleredema who were admitted to PUMC Hospital from 1982 to 2008 were retrospectively analyzed., Results: Neck and upper back lesions were detected in all patients, but no hand or foot involvement was noted. Among 54 patients who received serum immunoglobulin examination, 19 (35.2%) showed abnormal serum immunoglobulin results. Among 67 patients, 22 (32.8%) had concomitant diabetes mellitus. Alcian blue staining was performed in 35 patients, among whom 23 (65.7%) had positive results and 12 (34.3%) had negative results., Conclusions: Scleredema may have systemic involvements in addition to skin lesions. Patients with scleredema also tends to have concomitant diabetes mellitus. Alcian blue staining is not sufficient to differentiate scleredema and scleroderma.
- Published
- 2009
41. Board stiff.
- Author
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Mattei P, Templet J, and Cusack C
- Subjects
- Administration, Topical, Adult, Clobetasol administration & dosage, Glucocorticoids administration & dosage, Humans, Injections, Subcutaneous, Scleredema Adultorum drug therapy, Treatment Outcome, Triamcinolone administration & dosage, Neck, Scleredema Adultorum diagnosis
- Published
- 2007
- Full Text
- View/download PDF
42. [Nonpitting, livid induration of the skin].
- Author
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Kirschke J, Altmeyer P, and Kreuter A
- Subjects
- Humans, Male, Middle Aged, Scleredema Adultorum diagnosis
- Published
- 2007
- Full Text
- View/download PDF
43. Two cases of diabetic scleredema that responded to PUVA therapy.
- Author
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Nakajima K, Iwagaki M, Ikeda M, and Kodama H
- Subjects
- Diagnosis, Differential, Female, Humans, Male, Middle Aged, Scleredema Adultorum pathology, Diabetes Mellitus, Type 2, PUVA Therapy, Scleredema Adultorum diagnosis, Scleredema Adultorum drug therapy
- Published
- 2006
- Full Text
- View/download PDF
44. [Buschke Scleredema, case report].
- Author
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Foti R, Leonardi R, Fichera G, Di Gangi M, Leonetti C, Gangemi P, and De Pasquale R
- Subjects
- Adrenal Cortex Hormones therapeutic use, Aged, Biopsy, Colchicine therapeutic use, Diagnosis, Differential, Drug Therapy, Combination, Humans, Male, Scleredema Adultorum diagnosis, Scleredema Adultorum drug therapy, Treatment Outcome, Tubulin Modulators therapeutic use, Scleredema Adultorum pathology, Skin pathology
- Abstract
Buschke Scleredema is a rare connective tissue disorder of unknown aetiology, characterized by thickening of the dermis whose characteristics may mainly to mime systemic sclerosis, eosinophilic fasciitis and cutaneous amyloidosis. Scleredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. Scleredema can be classified into three clinical groups; each has a different history, course, and prognosis. Each one of these share reduction in chest articular movements and limitation of limbs movements. The skin histology is characterised by thickened dermis and increased spaces between large collagen bundles due to increased deposition of mucopolysaccharide in the dermis. Differential diagnosis can be made considering the typical clinical features and the histologic peculiarity. No therapy has been found effective. The authors describe a case of Buschke Scleredema successfully treated by steroids and colchicine. Clinical evaluation of skin induration and thickness as well as ultrasonography were performed at baseline and after treatment.
- Published
- 2006
- Full Text
- View/download PDF
45. Scleredema adultorum of Buschke: a case report and review of the literature.
- Author
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Beers WH, Ince A, and Moore TL
- Subjects
- Adult, Biopsy methods, Blood Protein Electrophoresis, Bone Marrow Examination, Humans, Hypergammaglobulinemia etiology, Lymphoma, B-Cell diagnosis, Lymphoma, B-Cell drug therapy, Male, Scleredema Adultorum diagnosis, Scleredema Adultorum drug therapy, Lymphoma, B-Cell complications, Scleredema Adultorum complications, Skin pathology
- Abstract
Objectives: To present a case of scleredema adultorum of Buschke associated with hypergammaglobulinemia and review the literature pertaining to this disease., Methods: Search of MEDLINE (PubMed) was performed using the words "Scleredema Adultorum." Only cases in the English language, with pertinent clinical information for analysis, were included., Results: We present a case of scleredema associated with hypergammaglobulinemia. The skin findings had been progressive for 2 years before he was diagnosed with scleredema. Our patient was found to have a B-cell lymphoma before being diagnosed with scleredema. The progression of skin thickening halted with no apparent correlation to immunosuppressive therapies or chemotherapeutic agents. A total of 165 cases of scleredema adultorum are described in the literature. There are 3 types of scleredema adultorum. Type 1 is usually preceded by a febrile episode and resolves spontaneously. Type 2 is associated with developing paraproteinemias including multiple myeloma. Type 3 is associated with diabetes mellitus., Conclusions: Scleredema adultorum of Buschke is a rare disorder which is sometimes associated with hypergammaglobulinemia; our patient had a B-cell lymphoma. Physicians should suspect scleredema in any patient with diffuse skin thickening where the hands and feet are spared, particularly if diabetes or a preceding febrile episode were present. Once the diagnosis of scleredema is made, evidence for the presence of hypergammaglobulinemia should be sought.
- Published
- 2006
- Full Text
- View/download PDF
46. [Scleredema of Buschke. A case report].
- Author
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Triki S, Fazaa B, El Fekih N, Zermani R, and Kamoun MR
- Subjects
- Female, Follow-Up Studies, Humans, Middle Aged, Skin pathology, Time Factors, Scleredema Adultorum diagnosis, Scleredema Adultorum pathology
- Abstract
Scleredema of Buschke is a rare disease whose aetiology remains unknown. It is characterised by symetric skin induration of the upper part of the body which usually resolve spontaneously. It can be associated with extra-cutaneous symptoms. We report a new case in a 55 year old women.
- Published
- 2006
47. Scleredema and diabetic sclerodactyly.
- Author
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Gruson LM and Franks A Jr
- Subjects
- Adult, Diagnosis, Differential, Hand Dermatoses complications, Hand Dermatoses drug therapy, Hand Dermatoses pathology, Humans, Male, Scleredema Adultorum diagnosis, Scleredema Adultorum drug therapy, Skin pathology, Diabetes Mellitus, Type 1 complications, Scleredema Adultorum pathology
- Abstract
A 40-year-old man presented with hardening of the skin of his hands and upper back, which had slowly worsened with time. His medical history included insulin-dependent diabetes mellitus since childhood. Histopathologic features of a biopsy specimen from the skin of his back showed a thick reticular dermis with collagen bundles in a haphazard array, which were separated by increased deposits of connective-tissue mucin. Scleredema and diabetic sclerodactyly are both well recognized skin findings that may occur in patients with diabetes mellitus. It is important to differentiate this condition from scleroderma. Treatment is difficult, and therefore many modalities have been used. This patient has improved with aminobenzoate, colchicine, and DMSO gel.
- Published
- 2005
48. Scleredema of Buschke in pediatric age group.
- Author
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Nagi A and Memon IA
- Subjects
- Child, Child, Preschool, Female, Humans, Scleredema Adultorum therapy, Scleredema Adultorum complications, Scleredema Adultorum diagnosis
- Abstract
Two cases of scleredema of Buschke are described, which occurred in pediatric age group--an uncommon occurrence after febrile illness. Both cases were self-limiting. Characteristic features are described.
- Published
- 2005
- Full Text
- View/download PDF
49. Buschke's scleredema and concomitant diseases: report of five cases and literature review.
- Author
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Lipozencić J, Marasović D, and Barisić-Drusko V
- Subjects
- Adult, Aged, Female, Humans, Male, Middle Aged, Scleredema Adultorum diagnosis, Scleredema Adultorum pathology
- Abstract
Scleredema adultorum or Buschke's scleredema is a rare disorder that belongs to the group of mucinoses. Diffuse, sudden swelling, hardening and induration of the skin can occur in children and younger women as well as in older men. The dermis is thickened because of the increased collagen glycosylation, like that in diabetic stiff skin syndrome. The face is most often involved. In older persons the trunk is usually first involved. There is relative sparing of the extremities, with no distal or Raynaud's phenomenon. Eosinophilic fasciitis, scleromyxedema, associated gammopathy, and other forms of edema and mucin deposition must be excluded on differential diagnosis. Antibiotics, sometimes high doses of intravenous penicillin, systemic corticosteroids, systemic PUVA and PUVA bath therapy seem most promising for the management of the disorder. Our patients were aged 27, 60, 64, 69 and 72 years, with typical skin lesions, thus that term "adultorum" does not appear to fit well.
- Published
- 2005
50. Radiotherapy of benign diseases-scleredema adultorum Buschke.
- Author
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Könemann S, Hesselmann S, Bölling T, Grabbe S, Schuck A, Moustakis C, De Simoni D, Willich N, and Micke O
- Subjects
- Humans, Male, Middle Aged, Practice Guidelines as Topic, Practice Patterns, Physicians', Treatment Outcome, Radiotherapy, Conformal methods, Scleredema Adultorum diagnosis, Scleredema Adultorum radiotherapy
- Abstract
Background: Scleredema adultorum Buschke is a rare disorder characterized by thickening of the dermis of the neck, head and upper trunk. Its etiology is unknown, but there may be a preceding history of infection and there is a known association with diabetes mellitus. Women are more frequently affected. Usually, the disease is self-limiting but some patients show progressive disease. In these cases therapeutic options are poor, with only case reports and small series supporting their use., Case Report: A 58-year-old patient with a scleredema of the neck and upper trunk is described, who was treated twice within 6 months by electron-beam radiation therapy. After the second course his symptoms improved significantly. A review of the literature of radiation treatment of this disease is given., Conclusion: . Regardless of the possible mechanisms in pathogenesis and treatment of scleredema adultorum Buschke, the application of ionizing radiation is an important, effective and well-tolerated therapy option in the treatment of severe cases and may candidate as the first-line treatment of this disease.
- Published
- 2004
- Full Text
- View/download PDF
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