Search

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.

Cutaneous mucinoses are a heterogenous group of conditions, characterized by the deposition of glycosaminoglycans (mucin) in the dermis, follicles, or in the epidermis. Major cutaneous mucinoses include lichen myxedematosus, scleredema, mucinoses associated with thyroid disease, reticular erythematous mucinosis, papulonodular mucinosis associated with connective tissue diseases, and cutaneous focal mucinosis. The aim of this review is to provide an update of what has currently been reported in the last 30-year literature about several new or emerging conditions of acquired cutaneous mucinoses in adults. Two new clinico-pathologic entities have been described: (i) Obesity-associated lymphedematous mucinosis and pretibial stasis mucinosis; (OACM) (ii) Nodular mucinosis of the breast (NMB). Two relatively new disease categories encompassing cutaneous mucinoses with a common pathogenetic mechanism have been identified: (i) Cutaneous mucinoses associated with drug exposure including biologic therapy, anti-colony-stimulating factor 1 receptor (CSF1R) and subcutaneous intralesional interferons (toxic dermal mucinoses); (ii) Cutaneous mucinosis following physical agents including mechanical traumas and after knee replacement.

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 P P = 0.021, P P P 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.

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.A diabetes mellitus é uma doença crónica, com uma prevalência crescente a nível mundial. As complicações da diabetes são uma causa major de morbilidade e mortalidade, condicionando custos importantes na área da saúde. Neste artigo é efetuada uma revisão das manifestações cutâneas mais frequentes presentes em doentes com diabetes e pré-diabetes. O reconhecimento atempado destas entidades é fulcral, levando não só a uma avaliação atempada do perfil metabólico como a uma referenciação e tratamento precoces. Desta forma, é possível minimizar os efeitos secundários da diabetes a longo prazo, melhorando significativamente o prognóstico dos doentes.

Background Scleredema is a rare sclerodermoid skin condition characterized by diffuse symmetrical thickening of the upper part of the body. Its association with monoclonal gammopathy and myeloma was recently described; very few cases have been reported to date. Case presentation A 66-year-old Sri Lankan woman who had been followed in a dermatology unit for 34 years with diffuse systemic sclerosis presented with an acute exacerbation of the skin disease. Absence of Raynaud’s phenomenon; sclerodactyly; characteristic lung, gastrointestinal, and cardiac involvement of systemic sclerosis; and repeatedly negative antinuclear antibodies test results led to reevaluation for the possibility of scleredema. Skin biopsies from four body sites showed normal epidermis and thickened reticular dermis with swollen collagen bundles separated from one another by clear spaces, resulting in fenestration. The skin appendages were not atrophied or bound down. Alcian blue staining showed interstitial mucin deposition. Serum protein electrophoresis demonstrated an abnormal monoclonal band in the β-region with a paraprotein level of 8.9 g/dl. Immunofixation showed an abnormal band in the γ-region consisting of immunoglobulin A and κ. Bone marrow biopsy revealed abnormal monoclonal plasma cells (15%) with multinuclearity. There was no evidence of end organ damage, and whole-body magnetic resonance imaging did not reveal any evidence of bone involvement. The patient’s diagnosis was revised as scleredema type 2 associated with IgA-κ, and she was referred to a hemato-oncologist for chemotherapy, which led to significant improvement in the skin condition. Conclusions Scleredema is a rare disorder that has an enigmatic, rare association with monoclonal gammopathy. Dermatologists should be aware of this rare but important association.

Hyperglycemia is defined by excess blood glucose and, when persistent, may lead to prediabetic and diabetic states. Insulin is a hormone produced by the beta cells of the pancreas in response to elevated blood glucose. Dysregulated insulin secretion or clearance results in hyperinsulinemia, which also is closely associated with type 2 diabetes mellitus (T2DM) and metabolic disturbances. Hyperglycemia and hyperinsulinemia are endemic within the United States and impart considerable morbidity and mortality. Cutaneous manifestations of chronic hyperglycemia and hyperinsulinemia include acanthosis nigricans (AN), diabetic dermopathy (DD), scleredema diabeticorum (SD), ichthyosiform skin changes, acrochordons, and keratosis pilaris (KP). Necrobiosis lipoidica (NL), bullosis diabeticorum (BD), and generalized granuloma annulare (GA) are more rarely reported in association with hyperglycemia and hyperinsulinemia; however, the strength of these associations remains unclear. It is crucial for dermatologists to recognize these cutaneous manifestations, as they may be the first signs of metabolic syndrome and insulin resistance. Early identification and management of these conditions is key to improving patient health outcomes and reducing health care costs. Herein, we review the clinical presentations of these conditions and their underlying pathophysiologic mechanisms.

Scleredema adultorum of Buschke is a rare skin disease characterized by skin thickening and tightening typically at the neck and the upper part of the body. This thickening results from increased mucin deposition in the reticular dermis. Three variants are recognized. Scleredema diabeticorum is one subtype associated with diabetes mellitus. We report a man with a history of poorly controlled diabetes presenting with extensive scleredema adultorum of the trunk in a "cuirasse" pattern associated with restrictive lung disease. Cutaneous ultrasonography revealed a marked thickening of the dermis as well as various echogenic spots in the dermis. Cutaneous hardness secondary to scleredema may cause limited mobility. Therefore, respiratory manifestations may be associated and assessed, mainly in cases of extensive scleredema involving trunk and shoulders. Moreover, cutaneous ultrasonography may be useful to monitor the evolution of scleredema and confirm the diagnosis.

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.

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.

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.

Sclerosing skin manifestations are more a symptom than a diagnosis and must therefore be meticulously clarified. Systemic scleroderma as a multiorgan disease must be distinguished from localized scleroderma or morphea because in addition to a different clinical picture they have a different prognosis and necessitate other therapeutic procedures. Rare sclerosing skin diseases with implications for internal medicine are eosinophilic fasciitis, Buschke's scleredema adultorum, scleromyxedema and nephrogenic systemic fibrosis.

Scleredema Adultorum Buschke is a disorder manifesting indurations of the skin mostly followed by musculoskeletal impairment. Data regarding this fact are seldom found and documentation of functional outcome of physical therapies and modalities related to Scleredema Adultorum Buschke is fragmentary. The aim of this case report is to demonstrate and to document an effective concept of rehabilitation in a patient suffering from Scleredema Adultorum Buschke.A treatment plan was developed containing therapeutic ultrasound, manual lymphatic drainage, and physiotherapy. Assessments were performed at baseline and after therapy.Treatment by physical therapies of presented patient resulted in an improved functionality. Five out of eight Short Form-36 questionaire sections increased in terms of enhanced general health and level of activity.Musculoskeletal impairment in a patient suffering from Scleredema Adultorum Buschke can be reduced by a multimodal concept of rehabilitation. Implications for Rehabilitation Rehabilitation professional should suspect scleredema in patients with diffuse skin thickening where hands and feet are spared Essential reactivating physical activity should be supported by skin softening physical modalities irrespective of etiology or primary therapy. There is a need for functional outcome measures and documentation in the rehabilitation of Scleredema Adultorum Buschke.

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.The different disease entities that can lead to dermal fibrosis should be differentiated. An earlier diagnosis of SSc would therefore be facilitated.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.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.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.

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.

The link between the metabolic syndrome (MetS) and skin diseases is increasingly important, with new associations being discovered. The association between MetS and psoriasis or MetS and hidradenitis suppurativa is well known, although the relationship between MetS and various autoimmune or inflammatory diseases has only recently attracted interest. Some inflammatory skin diseases, such as vitiligo, scleredema, recurrent aphthous stomatitis, Behcet disease, rosacea, necrobiosis lipoidica, granuloma annulare, skin tags, knuckle pads, and eruptive xanthomas, have possible associations with MetS. In this review, we examine the state of knowledge involving the relationship between MetS and these dermatologic diseases.

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.

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.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.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.

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.