Scleroedema

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 consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis...

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...

Immune checkpoint inhibitors (ICI) are the standard of care for various malignancies and have been associated with a wide spectrum of complications that are phenotypically akin to primary autoimmune diseases. While the literature on these toxicities is growing, there is a paucity of data regarding ICI-associated scleroderma which can carry significant morbidity and limit the ability to continue effective ICI therapy. Our review aimed to analyze the current literature on ICI-associated systemic scleroderma (ICI-SSc) and key scleroderma mimics...

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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...

Scleroedema is a rare clinical condition characterised by diffuse woody induration of skin commonly associated with diabetes mellitus, infections and monoclonal gammopathy. Its association with ovarian malignancy has not been reported. We report a case of a 56-year-old female with rapidly progressing skin thickening of limbs, face and trunk for 1 year and abdominal distension for 3 months. Patient had thickened skin, mask-like facies and ascites on examination. Atypical cells were seen in ascitic fluid. Contrast-enhanced computerised axial tomography scan of abdomen was suggestive of ovarian malignancy...

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...

INTRODUCTION: Diabetes mellitus and the prediabetic state are associated with a number of skin manifestations. This study is a systematic review of the following manifestations: acanthosis nigricans (AN), skin tags (ST), diabetic dermopathy (DD), rubeosis faciei (RF), pruritus (PR), granuloma annulare (GA), necrobiosis lipoidica (NL), scleroedema diabeticorum (SD) and bullosis diabeticorum (BD). These conditions possibly relate to underlying diabetogenic mechanisms. Our aim was to determine whether skin signs are feasible as cutaneous markers for the prediabetic or diabetic state...

BACKGROUND: Scleroedema adultorum Buschke (SB) is a rare disease involving scleroedema of the neck and shoulders. It can extend to the rest of the trunk and the limbs but characteristically spares the extremities. Three types of SB are distinguished: the first is acute and develops after an infectious disease, the second is of insidious evolution and is associated with monoclonal gammopathy, and the third is associated with type 2 diabetes. PATIENTS AND METHODS: We report the case of a type 2 diabetic patient presenting with progressive, oedematous timbering of the trunk associated with impaired mobility, dysphagia and restrictive respiratory syndrome...

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AIMS: To analyse systematically therapy-induced lesions of haematopoiesis in chronic idiopathic myelofibrosis (IMF). METHODS AND RESULTS: A total of 759 sequential bone marrow (BM) biopsies (median interval 32 months) were performed in 261 patients with IMF. Besides a control group (symptomatic treatment), monotherapies included busulfan, hydroxyurea and interferon. In all therapy groups hypoplasia of varying degree was a frequent finding and often accompanied by a patchy distribution of haematopoiesis...

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A 3-year-old Japanese girl with scleroedema was reported. She had had no signs of diabetes but did have a preceding bacterial infection in the tonsils three weeks before the skin lesion appeared. The skin on the face, shoulders, extensor aspect of the upper arms, and proximal half of the forearms was indurated. The skin lesions expanded from the middle part of the forearms to the wrists during the observation period. Thereafter, the induration gradually disappeared. A literature review revealed there were only six reports of scleroedema in children under 15 years old before 1996 in Japan; a total of 166 cases of the disease was reported in the same period...

Scleroedema adultorum Buschke is characterized by progressive hardening of the skin. In contrast to scleroderma the hardening occurs in the skin of the trunk while extremities remain largely free. Internal organs are said not to be involved in scleroedema adultorum Buschke. The full picture of the persistent form of scleroedema adultorum Buschke was observed in two patients. One patient complained of increasing dysphagia with regurgitation and aspiration. Manometry and X-ray cinematographic investigation showed inappropriate relaxation of the upper oesophageal sphincter...

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