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cleredema of Buschke is a rare pathological disorder of connective tissue, which is characterized by a woody, diffuse induration of the skin, most often in the upper extremities. We report an extremely rare complication of post-streptococcal infection in a six-year-old male complaining of gradually progressing, painless skin thickening and tightness which was preceded by a one-month history of fever, cough, and tonsillitis. By reporting this case, we hope to contribute to the creation of a database for future research aimed at better understanding the incidence, pathophysiology, and management of this extremely rare complication.

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., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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 .

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.

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.

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., (© 2017 Wiley Periodicals, Inc.)

Background: Body swelling in a child is a common symptom. Apart from systemic causes like renal, hepatic, and cardiac, rarely such a swelling may be caused by dermatologic conditions., Case Presentation: A child presented with swelling of the body which was subsequently diagnosed as scleredema, a rare and benign dermatologic condition. Scleredema can be confused with similar sounding terms like scleroderma and scleromyxedema., Conclusions: The case is presented to highlight scleredema as a rare cause of body swelling in paediatrics and to differentiate it from similar sounding rare terms like scleroderma and scleromyxedema.

Scleredema adultorum is a rare condition characterised by progressive collagen and mucin deposition in the skin. While the aetiology has not been clearly delineated, the condition is often associated with common infections. The current report describes a previously healthy 16-year-old boy who presented with 3 weeks of progressive neck swelling and skin induration. He had evidence of both active streptococcal and Ebstein-Barr virus (EBV) infections. Skin biopsy confirmed the diagnosis of scleredema. The patient was treated for his streptococcal infection, but otherwise managed conservatively. Clear improvement in the signs and symptoms was seen at a 3-month follow-up appointment. Scleredema can be a complication of streptococcal infection but to our knowledge has not been reported in association with EBV. It should be considered in the differential diagnosis of any patient presenting with cutaneous/subcutaneous induration and swelling of the face and/or neck.

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.

Scleredema diabeticorum is an uncommon condition. It is one of the cutaneous manifestations in diabetes mellitus that mainly occurs in obese middle-aged men with insulin-resistant diabetes. This condition is generally recalcitrant to therapy. Various treatments have been tried with inconsistent results. Here, we describe two cases of scleredema diabeticorum with substantial clinical improvement from a course of medium dose (60 J/cm²) ultraviolet A1 radiation therapy., (© 2010 Japanese Dermatological Association.)

Scleredema of Buschke (SB) is a rare disorder of connective tissue, characterized by hardening of the skin. Of unknown etiology, it may be associated with viral or bacterial infections, hematological alterations and diabetes mellitus (DM). In half of the reported cases of SB, there is association with DM. The most common characteristics among these patients are: being male adults, having long-term DM, bad metabolic control and presence of specific complications of DM. The histopathological examination shows dermal thickening, with widened collagen fibers separated by non-colored spots, which correspond to mucopolysaccharides deposits. The clinical consequences are: decrease in motility of the shoulders and an impairment of respiratory function. Several treatments are suggested in the literature, but with inconstant results. A case of SB is reported in a type 2 diabetes patient.

A 19-year male presented with acute onset, gradually progressive symmetric indurations involving the skin over the face, neck, shoulders and upper part of chest following an upper respiratory tract infection. Detailed history and examination did not reveal evidence of Raynauds' phenomenon, nail changes, digital ulcers, pigmentation or any systemic involvement. Autoantibodies for systemic sclerosis were absent. Histopathology of skin biopsy documented scleredema. Antistreptolysin O (ASO) titer was elevated. We diagnosed a case of Scleredema adultorum of Buschke following a streptococcal throat infection. We report this case to highlight the importance of clinically differentiating this relatively benign, self-limiting disorder from systemic sclerosis.

Background: The efficacy and safety of UVA1 (340-400 nm) phototherapy were established by studies from European countries., Purpose: Evaluate experience with UVA1 phototherapy for patients with cutaneous diseases in the United States., Methods: A retrospective analysis of 92 cases of UVA1-treated cutaneous conditions from four medical centers in the United States was performed., Results: Two-third of the patients showed a fair to good response (26-100% improvement) and one-third of the patients showed a poor response (0-25% improvement). Diseases with a moderate to good response (51-100% improvement) included scleredema adultorum, hand or foot dermatitis, atopic dermatitis, morphea (medium or medium- to high-dose UVA1), systemic sclerosis, and urticaria pigmentosa. Besides tanning, other adverse effects were found in 15% of patients, which include pruritus, erythema, tenderness, and burning sensation. Patients with skin types I-III responded better that those with a darker skin type., Conclusion: UVA1 phototherapy is a useful and well-tolerated treatment option for a variety of skin conditions.

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.

A 41 year male, diabetic on treatment presented with persistent erythema and. "taut" skin over neck and back since 1 year. The lesions showed symmetric and gradual progression. There was no history of prior sore throat. On examination effected skin was erythematous, woody hard and unpinchable. Scleredema diabeticorum (diutinum), although sharing clinical and historical features with Scleredema adultorum has no prodromal infection, is more extensive and affected individuals are characteristically obese, middle aged diabetics who often have accompanying microangiopathies and macroangiopathies. Recognition of scleredema by the physician has prognostic and therapeutic implications in the management of the coexisting diabetes.

Scleredema is a rare disease characterized by extensive cutaneous woody, non-pitting induration that spreads throughout the body. We describe a patient with scleredema associated with paraproteinaemia who was successfully treated by extracorporeal photopheresis.

Scleredema adultorum is a rare connective tissue disorder reported usually following streptococcal infection, influenza, measles, and mumps. It has been reported occasionally following trauma and tuberculous lymphadenitis. This is a report of scleredema adultorum developing after chicken pox in an eight-year-old male child. The diagnosis was established by characteristic picture on skin biopsy using special stain. The patient had a benign course and a spontaneous recovery in two weeks. The case has been reported as the first case of scleredema adultorum developing after chicken pox.

We report a 49-year-old woman with severe diabetic scleredema (DS). The patient had non-insulin-dependent diabetes mellitus (NIDDM) for 9 years and noticed thickened skin on her back 3 years previously. Her DS rapidly extended to her back and extremities with pain and immobility. Her symptoms of DS improved dramatically after establishing strict glycemic control and intravenous administration of prostaglandin E1 (PGE1). However, the histological findings of her skin biopsy did not change even after the treatment for 12 weeks, and her symptoms worsened again after discontinuation of glycemic control and PGE1 treatment. The causes of DS have been considered to be metabolic abnormalities associated with hyperglycemia and hypoxia in the skin due to diabetic microangiopathy. PGE1 was an effective treatment for DS in our patient. Strict control of hyperglycemia and PGE1 treatment may be sufficient to manage DS, although a very long treatment period is necessary.

We report a patient with the typical lesions of diabetic scleredema. Histological findings of the involved skin were thickening of the dermis, depositions of mucins, and fibrosis. Biochemical analysis revealed an increase in glycosaminoglycans in the involved skin as well as in the cutaneous lupus mucinosis. Mucinous materials were composed of hyaluronic acid.

We describe a patient with insulin-dependent diabetes without vascular complications in whom scleredema was confined to the thighs. Electron microscopy demonstrated heterogeneity in both the size and density of the collagen fibrils and the presence of filamentous material between them.

Skin manifestations associated with diabetes mellitus are common and, with close scrutiny, can occur in most, if not all, patients. Little information is available on what common pathophysiologic thread is responsible for the skin manifestation and diabetes. Although controlled studies are lacking, a recognition of the skin manifestations, treatment, and prognosis will help patients and their caregivers to understand treatment alternatives more clearly.

A 21-year-old housewife with maturity onset type diabetes of young people developed scleredema diabeticorum, scleroderma-like skin thickness on forearms and dorsum of hands, digital sclerosis and cheiroarthropathy. She had diabetes mellitus since the age of 11 years. Her grandfather on the mother's side, her mother and 3 of 5 her mother's brothers and sisters have diabetes mellitus. Blood glucose was 295 mg/dl. Urinary glucose was 5.3 g/day. Nail fold capillary microscopy revealed a progressive systemic sclerosis pattern. Histologically, hematoxylin and eosin sections from back and forearm skin demonstrated broad collagen bundles separated by widened clear spaces throughout the thickened dermis.

Numerous skin disorders or skin lesions are associated with a monoclonal gammopathy (formerly: paraproteinemia). These skin diseases can be differentiated in the following way: specific skin lesions caused by monoclonal immunoglobulin producing cells in the skin, in generalized plasmocytoma or morbus Waldenström. In another group of disorders the monoclonal immunoglobulins induce functional disturbances, e.g. Raynaud syndrome (if the monoclonal immunoglobulins are cryoglobulins), xanthomatosis through interactions between these immunoglobulins and lipoproteins, purpura in immunoglobulin induced stasis and hyperviscosity. The unlimited spreading of the plasmocytoma cells replaces normal immunoglobulin producing plasma cells followed by a disturbance of the humoral immune response, which is frequently accompanied by atypical infections of skin or other organs. Furthermore, an almost obligatory association with monoclonal gammopathies has been demonstrated in a few skin diseases, e.g. scleromyxedema. However, the role of the immunoglobulins in these disorders is unknown. In some other skin diseases, such as pyoderma gangrenosum, acrodermatitis atrophicans Herxheimer, scleredema Buschke etc., a monoclonal gammopathy is only detectable in some patients.