RESUMO
Diabetes mellitus is a significant worldwide health concern and cutaneous manifestations are common. This review describes characteristic skin findings of diabetes, general skin findings related to diabetes, and findings related to diabetes treatment with a focus on clinical presentation, diagnosis, pathophysiology, epidemiology, and treatment. As the prevalence of diabetes continues to rise, cutaneous manifestations of diabetes mellitus likely will be encountered more frequently by physicians in all disciplines including dermatologists and primary care physicians. Accordingly, knowledge regarding the prevention, diagnosis, and management of cutaneous manifestations is an important aspect in the care of patients with diabetes.
Assuntos
Complicações do Diabetes/patologia , Diabetes Mellitus/epidemiologia , Dermatopatias/diagnóstico , Dermatopatias/prevenção & controle , Dermatopatias/fisiopatologia , Acantose Nigricans/etiologia , Acantose Nigricans/patologia , Acantose Nigricans/terapia , Dermatologistas/estatística & dados numéricos , Pé Diabético/etiologia , Pé Diabético/patologia , Pé Diabético/terapia , Saúde Global/estatística & dados numéricos , Humanos , Conhecimento , Lipodistrofia/etiologia , Lipodistrofia/patologia , Lipodistrofia/terapia , Pessoa de Meia-Idade , Necrobiose Lipoídica/etiologia , Necrobiose Lipoídica/patologia , Necrobiose Lipoídica/terapia , Médicos de Atenção Primária/estatística & dados numéricos , Prevalência , Escleredema do Adulto/etiologia , Escleredema do Adulto/patologia , Escleredema do Adulto/terapia , Dermatopatias/epidemiologiaAssuntos
Tratamento por Ondas de Choque Extracorpóreas/métodos , Escleredema do Adulto/terapia , Humanos , Masculino , Pessoa de Meia-Idade , Amplitude de Movimento Articular , Escleredema do Adulto/diagnóstico por imagem , Escleredema do Adulto/fisiopatologia , Ombro/fisiopatologia , Resultado do TratamentoRESUMO
BACKGROUND: Scleredema adultorum (Buschke's scleredema) is a cutaneous mucinosis of unknown origin, clinically characterized by a diffuse induration of the skin usually involving the neck, shoulders and back, which limits patients' mobility. CASE REPORT: We report a case of a 50-year-old woman who presented a chronic sclerodermiform syndrome for 2 years associated with type 1 diabetes. Physical examination revealed an extensive skin induration involving the shoulders, neck and back. Histologic examination confirmed the diagnosis of scleredema adultorum. The patient was treated with extracorporeal photopheresis (EPP) twice a month for two months. At follow-up, mobility was highly improved after two months. Beneficial effect of EPP was maintained on the long term while sessions were spaced. DISCUSSION: EPP is an unconventional treatment of Buschke's scleredema. We described a case of Buschke's scleredema successfully treated with EPP which may represent a therapeutic option for the treatment of scleredema.
Assuntos
Fotoferese/métodos , Escleredema do Adulto/terapia , Feminino , Humanos , Pessoa de Meia-Idade , Pele/patologiaRESUMO
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).
Assuntos
Dermopatia Fibrosante Nefrogênica/diagnóstico , Dermopatia Fibrosante Nefrogênica/terapia , Escleredema do Adulto/diagnóstico , Escleredema do Adulto/terapia , Escleromixedema/diagnóstico , Escleromixedema/terapia , Diagnóstico Diferencial , Humanos , Dermopatia Fibrosante Nefrogênica/patologia , Escleredema do Adulto/patologia , Escleromixedema/patologiaRESUMO
Scleredema is a deposition disorder which presents as diffuse, symmetric, non-pitting skin induration from dermal deposits of glycosaminoglycans ("mucins"). It classically affects the upper back and posterior neck, often causing skin tightness and decreased range of motion. In most patients the clinical course is chronic and treatment options are limited. We report a case of a patient diagnosed with scleredema associated with longstanding insulin dependent diabetes who was treated successfully with IVIg. Additionally, we have reviewed the literature reporting other patients with scleredema treated with IVIg.
Assuntos
Fármacos Dermatológicos/uso terapêutico , Complicações do Diabetes/terapia , Diabetes Mellitus Tipo 1/complicações , Imunoglobulinas Intravenosas/uso terapêutico , Escleredema do Adulto/terapia , Adulto , Progressão da Doença , Feminino , Humanos , Escleredema do Adulto/etiologia , Escleredema do Adulto/patologiaRESUMO
Scleredema adultorum, also known as scleredema of Buschke, is a rare connective tissue disease with unknown etiology, which is characterized by diffuse skin induration of face, neck, upper chest, back, shoulders and arms. Although there is no established treatment for this disease, the efficacy of phototherapy has been reported. We herein describe a case of scleredema adultorum successfully treated with narrow-band ultraviolet B and discuss a potential mechanism explaining its efficacy for fibrotic skin diseases.
Assuntos
Escleredema do Adulto/terapia , Terapia Ultravioleta/métodos , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
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.
Assuntos
Fármacos Dermatológicos/administração & dosagem , Diabetes Mellitus Tipo 2 , Metotrexato/administração & dosagem , Escleredema do Adulto/diagnóstico , Terapia Combinada , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade , Terapia PUVA , Escleredema do Adulto/terapiaRESUMO
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.
Assuntos
Imunoglobulina G/imunologia , Imunoglobulinas Intravenosas/uso terapêutico , Escleredema do Adulto/imunologia , Escleromixedema/imunologia , Idoso , Feminino , Humanos , Fatores Imunológicos/uso terapêutico , Masculino , Pessoa de Meia-Idade , Escleredema do Adulto/diagnóstico , Escleredema do Adulto/terapia , Escleromixedema/diagnóstico , Escleromixedema/terapiaAssuntos
Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Escleredema do Adulto/tratamento farmacológico , Infecções Estreptocócicas/complicações , Adulto , Antibacterianos/uso terapêutico , Anti-Inflamatórios , Feminino , Humanos , Metilprednisolona/uso terapêutico , Penicilina V/uso terapêutico , Escleredema do Adulto/patologia , Escleredema do Adulto/terapia , Infecções Estreptocócicas/tratamento farmacológico , Terapia UltravioletaRESUMO
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.
Assuntos
Eosinofilia/diagnóstico , Fasciite/diagnóstico , Dermopatia Fibrosante Nefrogênica/diagnóstico , Escleredema do Adulto/diagnóstico , Esclerodermia Localizada/diagnóstico , Escleromixedema/diagnóstico , Diagnóstico Diferencial , Eosinofilia/etiologia , Eosinofilia/terapia , Fasciite/etiologia , Fasciite/terapia , Humanos , Dermopatia Fibrosante Nefrogênica/etiologia , Dermopatia Fibrosante Nefrogênica/terapia , Escleredema do Adulto/etiologia , Escleredema do Adulto/terapia , Esclerodermia Localizada/etiologia , Esclerodermia Localizada/terapia , Escleromixedema/etiologia , Escleromixedema/terapiaRESUMO
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Assuntos
Masculino , Pessoa de Meia-Idade , Humanos , Escleredema do Adulto/complicações , Escleredema do Adulto/diagnóstico , Escleredema do Adulto/terapia , Clobetasol/uso terapêutico , Fotoquimioterapia/métodos , Fotoferese/métodos , Eritema Endurado/complicações , Eritema Endurado/diagnóstico , Faringite/diagnóstico , Derme/anatomia & histologia , Derme/citologia , Derme/microbiologia , Derme/patologia , Eritema Endurado/tratamento farmacológico , Eritema Endurado/patologia , Eritema Endurado/radioterapiaRESUMO
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.
Assuntos
Escleredema do Adulto/complicações , Escleredema do Adulto/diagnóstico , Criança , Pré-Escolar , Feminino , Humanos , Escleredema do Adulto/terapiaRESUMO
El escleredema de Buschke se caracteriza por un engrosamiento de la dermis con depósito de mucina entre las fibras de colágeno, que se manifiesta como un endurecimiento de la piel, predominantemente en la mitad superior del tronco. Se ha relacionado con diabetes mellitus de larga evolución y mal controlada, gammapatías monoclonales e infecciones estreptocócicas. Se estudiaron los pacientes con el diagnóstico clínico de escleredema confirmado mediante estudio histopatológico y asociado a diabetes mellitus. Se revisaron las enfermedades asociadas, las características clínicas e histopatológicas, el curso evolutivo y la respuesta al tratamiento. El escleredema se caracteriza por un endurecimiento cutáneo que afecta a la mitad superior del tronco. En una gran proporción de casos se asocia a diabetes mellitus mal controlada, de curso prolongado durante años y con escasa respuesta al tratamiento
Scleredema of Buschke is characterized by a thickening of the dermis with mucin deposits among the collagen fibers, which manifests as a hardening of the skin, predominantly on the upper trunk. It has been associated with long-standing, poorly controlled diabetes mellitus, monoclonal gammopathies and streptococcus infections. We studied patients with a clinical diagnosis of scleredema confirmed by means of a histopathological study, and associated with diabetes mellitus. We reviewed associated diseases, clinical and histopathological characteristics, evolution and response to treatment. Scleredema is characterized by hardening of the skin, affecting the upper trunk. A large proportion of cases are associated with long-standing, poorly controlled diabetes mellitus, with little response to treatment
Assuntos
Masculino , Feminino , Adulto , Idoso , Humanos , Escleredema do Adulto/complicações , Escleredema do Adulto/diagnóstico , Escleredema do Adulto/terapia , Diabetes Mellitus/complicações , Diabetes Mellitus/terapia , Escleredema do Adulto/patologia , Escleredema do Adulto/fisiopatologia , Diagnóstico Diferencial , BiópsiaRESUMO
A 72-year-old man with diabetes-associated scleredema is presented. The patient had a long history of diabetes mellitus that had been difficult to control with complications of retinopathy, nephropathy, and arteriosclerosis leading to myocardial infarcts and stroke. The scleredema has remained stable with 4 months of topical clobetasol ointment twice daily and biweekly physical therapy. Diseases associated with scleredema and therapeutic options are summarized.
Assuntos
Escleredema do Adulto , Idoso , Anti-Inflamatórios/uso terapêutico , Clobetasol/uso terapêutico , Diabetes Mellitus Tipo 2/complicações , Humanos , Masculino , Modalidades de Fisioterapia , Escleredema do Adulto/complicações , Escleredema do Adulto/patologia , Escleredema do Adulto/terapiaAssuntos
Escleredema do Adulto/diagnóstico , Antibacterianos/uso terapêutico , Diabetes Mellitus Tipo 2/complicações , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade , Modalidades de Fisioterapia , Amplitude de Movimento Articular , Escleredema do Adulto/complicações , Escleredema do Adulto/terapiaRESUMO
The cutaneous mucinoses are a complex group of dermatologic diseases with local, follicular, or diffuse disease. The diffuse cutaneous mucinoses are remarkable not only for their dermal disease, but also for the numerous systemic manifestations. It is important that the clinical dermatologist be able accurately to diagnose and differentiate scleredema, scleromyxedema, REM, generalized myxedema of hypothyroidism, and pretibial myxedema of hyperthyroidism. Because of the variability of associated systemic manifestations, some with substantial morbidity and mortality, accurate diagnosis is vital for awareness and appropriate management.