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Acta Medica Portuguesa Jun 2019Diabetes is a serious, chronic disease with a rising prevalence worldwide. Its complications are a major cause of morbidity and mortality and contribute substantially... (Review)
Review
Diabetes is a serious, chronic disease with a rising prevalence worldwide. Its complications are a major cause of morbidity and mortality and contribute substantially to health care costs. In this article the authors review the most common and sensitive skin manifestations that can be present on patients with diabetes and prediabetes. The prompt recognition of these frequently underestimated entities is extremely important as it may trigger not only an adequate metabolic evaluation but also a timely referral and appropriate treatment, minimizing the secondary effects of long-term diabetes and improving the prognosis of diabetic patients.
Topics: Acanthosis Nigricans; Blister; Diabetes Complications; Diabetic Angiopathies; Diabetic Foot; Granuloma Annulare; Humans; Necrobiosis Lipoidica; Prediabetic State; Scleredema Adultorum; Skin Diseases; Skin Diseases, Infectious; Skin Diseases, Vascular; Xanthomatosis
PubMed: 31292028
DOI: 10.20344/amp.10738 -
Giornale Italiano Di Dermatologia E... Apr 2018Scleroderma is divided into a systemic form called systemic sclerosis and a localized form also called morphea. According to 2013 ACR/EULAR Classification Criteria for... (Review)
Review
Scleroderma is divided into a systemic form called systemic sclerosis and a localized form also called morphea. According to 2013 ACR/EULAR Classification Criteria for Systemic Sclerosis, developed by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR), skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for a patient to be classified as having scleroderma. Histological examination is not included in the diagnostic criteria and is not routinely performed. Skin biopsy is recommended only in the case of diagnostic doubt with other scleroderma like disorders (scleromyxedema, scleredema, nephrogenic systemic fibrosis). Alternatively, skin biopsy is also often performed for research purposes. Indeed, the first step analysis of new cytokines or pathways that may contribute to the pathogenesis of the disease requires the evaluation of their expression or activation in the skin of scleroderma patients compared to healthy controls. The histological picture of the skin in bot localized and systemic scleroder shows initially microvascular alterations and chronic inflammation while in the more advanced stages skin fibrosis prevails. Localized scleroderma (LS) or morphea includes a number of subtypes which are classified more according to their clinical presentation rather than histopathological pictures. However, some histopathologic changes may be useful in differentiating each entity from the others and from other sclerodermoid disorders.
Topics: Biopsy; Cytokines; Diagnosis, Differential; Humans; Scleroderma, Localized; Scleroderma, Systemic; Skin Diseases
PubMed: 29368844
DOI: 10.23736/S0392-0488.18.05922-9 -
Dermatology Reports Nov 2022Scleredema of Buschke is a rare connective tissue disease with a poorly understood pathogenesis. Three types of scleredema have been distinguished according to its...
Scleredema of Buschke is a rare connective tissue disease with a poorly understood pathogenesis. Three types of scleredema have been distinguished according to its association with preceding or underlying conditions. Type 1 is usually secondary to a febrile infection, type 2 is mostly associated with paraproteinemia and type 3, usually named scleredema diabeticorum, has a strict association with Diabetes mellitus. A diffuse, non-pitting swelling and induration of the skin define this disease. The skin histology is characterized by a normal or slightly thinned epidermis, and the dermis containing a decreased number of elastic fibers and thick large swollen collagen bundles separated by mucopolysaccharide deposits in the deep reticular dermis. In this report we present a 58-year-old man with scleredema diabeticorum controlled with a topical steroid cream and an optimization of glycemic control. We reviewed clinical, histopathological characteristics and the various possible treatments.
PubMed: 36483221
DOI: 10.4081/dr.2022.9477 -
Journal of Scleroderma and Related... Jun 2019Scleromyxedema is a rare fibromucinous disorders, with several clinical and pathological overlaps with scleroderma and scleredema. Etiopathogenesis remains uncovered,... (Review)
Review
Scleromyxedema is a rare fibromucinous disorders, with several clinical and pathological overlaps with scleroderma and scleredema. Etiopathogenesis remains uncovered, and no explanation has been provided either for the origin of mucin deposition or for the paraprotein role. The disease does not show gender predilection and affects mainly middle-age adults. The course is unpredictable, and prognosis remains guarded for renal, cardiac, and neurologic complications, especially in the setting of dermato-neuro syndrome. A valuable recent progress is the consensus definition of diagnostic criteria and lines of treatment, which hold the promise to improve the early recognition and management of this rare condition worldwide. High-dose intravenous immunoglobulin has been suggested as the first-line treatment either alone or associated with systemic steroids and/or thalidomide. In very recalcitrant cases, adjunctive bortezomib and/or autologous stem cell transplant might be considered. Melphalan treatment was associated with very toxic side effects and actually is no longer recommended.
PubMed: 35382389
DOI: 10.1177/2397198318824929 -
Rheumatic Diseases Clinics of North... Feb 2008Many conditions presenting with clinical hard skin and tissue fibrosis can be confused with systemic sclerosis (scleroderma). These disorders have very diverse... (Review)
Review
Many conditions presenting with clinical hard skin and tissue fibrosis can be confused with systemic sclerosis (scleroderma). These disorders have very diverse etiologies and often an unclear pathogenetic mechanism. Distinct clinical characteristics, skin histology, and disease associations may allow one to distinguish these conditions from scleroderma and from each other. A prompt diagnosis is important to spare patients from ineffective treatments and inadequate management. This article highlights nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy), eosinophilic fasciitis (Shulman's syndrome), scleromyxedema, and scleredema. These often are detected in the primary care setting and referred to rheumatologists for further evaluation. Rheumatologists must be able to promptly recognize them to provide valuable prognostic information and appropriate treatment options for affected patients.
Topics: Diagnosis, Differential; Fasciitis; Fibrosis; Humans; Scleroderma, Diffuse; Scleromyxedema; Skin
PubMed: 18329541
DOI: 10.1016/j.rdc.2007.11.001 -
Current Treatment Options in... Mar 2016Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin...
Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin involvement, presence or absence of systemic complications and unique associated laboratory abnormalities often help to distinguish between these diseases. It is imperative that an effort is made to accurately differentiate between scleroderma and its mimics, in order to guide long-term management and facilitate implementation of the appropriate treatment modality where indicated.
PubMed: 28473954
DOI: 10.1007/s40674-016-0038-7 -
Frontiers in Medicine 2017Sclerosing skin diseases represent a large number of distinct disease entities, which include systemic sclerosis, localized scleroderma, and scleredema adultorum. These... (Review)
Review
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.
PubMed: 28868289
DOI: 10.3389/fmed.2017.00120 -
Annals of Nutrition & Metabolism 2019Youth-onset type 2 diabetes (T2D) is increasing in many countries, creating large personal and societal burdens. While many primary health-care professionals (HCPs) are... (Review)
Review
BACKGROUND
Youth-onset type 2 diabetes (T2D) is increasing in many countries, creating large personal and societal burdens. While many primary health-care professionals (HCPs) are aware of the classic symptoms of T2D, there are several other manifestations that could indicate its presence.
SUMMARY
This narrative review summarizes information on these symptoms and indicators, focusing on those less well known. The classic symptoms and comorbidities include frequent urination, excessive thirst, metabolic syndrome, and obesity. In addition to these, the presence of dermatological (e.g., acanthosis nigricans, granuloma annulare, necrobiosis lipoidica diabeticorum, and scleredema), gynecological (e.g., polycystic ovary syndrome, oligomenorrhea, and vulvovaginitis), hepatological (e.g., nonalcoholic fatty liver disease), and psychiatric diseases (e.g., psychosis, depression, and autism) could indicate that a patient has T2D or is at increased risk of T2D. Other less well-known indicators include abnormal blood tests (e.g., oxidized lipids, inflammation markers, hepatokines, and adipokines), prescriptions for antipsychotic medications or statins, and disrupted sleep patterns. Key Message: Due to the diversity of T2D manifestations in young people, primary HCPs need to remain alert to its possible presence.
Topics: Adolescent; Diabetes Mellitus, Type 2; Diagnosis, Differential; Humans; Primary Health Care
PubMed: 31096219
DOI: 10.1159/000500234 -
Frontiers in Immunology 2023Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected... (Review)
Review
Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected skin biopsies described histopathological changes similar to those of scleroderma located strictly on the skin or those of systemic sclerosis. These skin lesions can be found in inflammatory diseases with autoimmune substrate (generalized morphea, chronic graft versus host disease, eosinophilic fasciitis), tissue storage diseases (scleredema, scleromyxedema, nephrogenyc systemic fibrosis, systemic amyloidosis), metabolic diseases (porphyrya cutanea tarda, phenylketonuria, hypothyroidism, scleredema diabeticorum), progeroid syndromes. Given the multiple etiologies of sclerodermal lesions, a correct differential diagnosis is necessary to establish the appropriate treatment.
Topics: Scleroderma, Systemic; Humans; Diagnosis, Differential
PubMed: 37600771
DOI: 10.3389/fimmu.2023.1180221