Scleredema Research Articles

Scleredema adultorum of Buschke during pregnancy

Scleredema adultorum of Buschke during pregnancy

A clinicoepidemiological study of pattern of skin disorders among diabetes mellitus in a tertiary care centre

Diabetes Mellitus (DM), a most common metabolic disorder affects every organ of the body including skin causing increased morbidity and serious impact on quality of life. Aim of this study is to analyse the prevalence and pattern of cutaneous manifestations among type 1 and type 2 DM correlating it with the mean duration of diabetes. This was an observational study conducted on 200 consecutive patients with DM attending dermatology department. Apart from detailed history, thorough clinical examination and relevant investigations were done. Skin biopsy were done in cases with diagnostic ambiguity. Cutaneous infections occurred in majority (70.5%). Among fungal infections (59.5%) candidiasis was the most common (28%). Pruritus and neuropathy were observed in 37% and 33% respectively and was more common in type 1 DM. Leg ulcer was observed in 8.5%. Vitiligo, oral lichen planus, granuloma annulare were seen common among type 1 DM. Psoriasis, acrochordon, acanthosis nigricans, cutaneous amyloidosis, bullous pemphigoid and diabetic bulla were common in Type 2 DM. Mucor mycosis, scleredema diabeticorum, cheiroarthropathy and perforating dermatoses were uniquely observed among prolonged uncontrolled diabetics. Candidiasis was found to be an early manifestation of diabetes. Poor glycemic control and longer duration of diabetes were associated with an increased frequency and number of skin disorders. Skin manifestations are more common among undiagnosed and uncontrolled diabetics..

Treatment of scleredema adultorum of Buschke with intravenous immunoglobulin and mycophenolate mofetil in a 14-year-old girl: a case report

BackgroundScleredema adultorum of Buschke is a rare disease characterized by firm and non-pitting edema of the skin. The condition is rare with unknown etiology. Diagnosis is made on the basis of clinical findings and skin biopsy.Case presentationHere, we describe a 14-year-old Iranian girl presenting with non-pitting edema and woody thickening of the skin that progressed within a month. She was evaluated for possible underlying malignancy or connective tissue disorders, which were excluded by multiple laboratory workups. She underwent a skin biopsy which confirmed the diagnosis of scleredema, and she was successfully treated with intravenous immunoglobulin and mycophenolate mofetil.ConclusionWhile scleredema adultorum of Buschke is a rare disease with no definite treatment, our effort through this report was to highlight the possible benefits of treatment by intravenous immunoglobulin and mycophenolate mofetil.

Differential diagnosis of sclerosing skin diseases: scleredema adultorum of Buschke

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 aclinical 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. Management of the disease includes physiotherapy, physical therapies such as ultraviolet (UV) or ionizing irradiation, intravenous immunoglobulins and interdisciplinary treatment directed at associated diseases. Optimizing diabetes therapy and thereby decreasing insulin use may confer significant improvement.

Buschke's Scleredema Post Covid 19 Infection: About a Case

Buschke scleredema is a rare disease characterized by sclerotic edema that affects the upper part of the trunk and can extend to the limbs. It occurs at any age. It can be accompanied by extracutaneous damage. Three types can be distinguished within this pathology: type I appears suddenly secondary to an infection. Type II develops chronically outside of an infectious and diabetic context associated with monoclonal gammopathy. Type III has been called diabetic scleredema.

FRI644 A Rare Case Of Autoimmune Polyglandular Disease Type 3, With Scleredema Adultorum Of Buschke, Type 1 Diabetes And Graves Disease

Abstract Disclosure: C. Pérez-Castañeda: None. G.A. González-Villaseñor: None. M. Mercado: None. A. Pozos-Ochoa: None. H.J. Medina: None. E. Gamez: None. M.T. Muñoz-Magallanes: None. Background: Scleredema Adultorum of Buschke is an uncommon skin disease, characterized by hardening and stiffness of the subcutaneous tissues, which typically starts at the neck; affecting the upper back causing immobilization; it may develop both in type 1 and type 2 diabetes mellitus and it is related with prolonged exposure to hyperglycemia, obesity and poor metabolic control. Treatment options include topical as well as systemic treatments and physical modalities; but therapy is often reported as unsuccessful. We present a case of scleredema of Buschke occurring in a patient with Autoimmune Polyglandular Disease Type 3 involving thyroid autoimmune disease and type one diabetes which to our knowledge it´s the first time to be described in literature. Clinical Case: A 42 year old male presented with history of a long standing type 1 diabetes and hypothyroidism after radioiodine treatment for refractory Graves´ disease. He complained of skin thickening and hardening in the nape, back and shoulder areas which has been slowly progressing over the last six years. Physical examination revealed a symmetric, painless, poorly defined non-pitting induration of the skin, with hyperpigmentation and peau d´ orange appearance, involving the back of the scalp, neck, upper back and both shoulders; at palpation there was a woody 1 hardness and thickness of the skin associated with failure of either rolling nor pinching the skin. There was a significant decreased range of motion in both shoulders. We performed an ultrasound which demonstrated an increased thickness of the dermis with a pronounced posterior acoustic shadow and presence of multiple echogenic dots. A magnetic resonance was subsequently conducted, revealing a thickening of the skin at subcutaneous tissue primarily through the interscapular region and a hyperintense band at the dermis. Biopsy specimen showed collagen bundles separated by gaps that correspond to mucopolysaccharide deposits. The patient was followed at the outpatient clinic, we initiated physiotherapy and we adjusted his diabetes treatment in order to achieve adequate control, the patient now refers a steady state on the clinical picture. Conclusion: Scleredema is a rare disease, treatment options include topical as well as systemic treatments and physical modalities; but therapy is often reported as unsuccessful, probably associated with the few cases published in the literature; so there is no standard therapeutic protocol. The course is usually unpredictable and the disease itself is refractory. This is the first case reporting the rare association of type one diabetes, Grave s disease and scleredema of Buschke. 2 Presentation: Friday, June 16, 2023

Immunologic and nonimmunologic sclerodermal skin conditions - 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.

Scleredema of Buschke in a little child and its successful treatment with ultraviolet A1 phototherapy

Scleredema of Buschke in a little child and its successful treatment with ultraviolet A1 phototherapy

Scleredema diabeticorum.

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.

Клинический случай развития В-клеточной лимфомы у пациентки с оверлап-синдромом первичного синдрома Шегрена и склередемы Бушке

Представлено описание клинического случая развития В-клеточной лимфомы у пациентки с оверлап-синдромом первичного синдрома Шегрена (ПСШ) и склередемы Бушке (СБ). Склередема Бушке — редкое заболевание, характеризующееся диффузным индуративным отеком и уплотнением кожи. Принята условная классификация этого заболевания в зависимости от этиологического фактора: тип 1 — ассоциированный с инфекционным заболеванием; тип 2 — с парапротеинемией и тип 3 — с сахарным диабетом. Существуют очень редкие случаи, связанные со злокачественными новообразованиями или аутоиммунными заболеваниями. Данный клинический случай описывает редкое сочетание СБ с аутоиммунным заболеванием — ПСШ, что подчеркивает важность изучения других ассоциаций СБ, в частности с аутоиммунными заболеваниями, для обеспечения надлежащего ведения и лечения таких пациентов. Заболевание у пациентки 51 года дебютировало с плотного отека лица, сухости кожи, синдрома «сухого глаза» и ощущения песка в глазах, повышения температуры до субфебрильных цифр и плотных образований кожи в области декольте, молочных желез и межлопаточной области, что патогистологически было расценено как СБ. Выявлены лимфаденопатия, ксерофтальмия, сиалоаденит, синдром Рейно, диагностировано поражение легких (альвеолит и бронхиолит), позитивные ANA, anti-SSa/La, anti-SSb/Ro. На основании данного симптомокомплекса пациентке был установлен диагноз «оверлап СБ с ПСШ». Применение комбинированной терапии метотрексатом и метилпреднизолоном позволило снизить активность заболевания, клинические и лабораторные проявления болезни. Однако через 9 месяцев после появления первых кожных симптомов возникло выбухание левого глаза, распространенная лимфаденопатия. При патогистологическом обследовании установлен диагноз неходжкинской В-клеточной лимфомы. Применение терапии ритуксимабом привело к значительному регрессу опухоли орбиты. Актуальность данного клинического наблюдения — не только в редкости сочетанной патологии, но и в необходимости проведения ранней диагностики развития лимфомы на фоне ПСШ, на основании факторов риска, к которым относят низкий уровень С4 и/или С3, увеличение околоушных слюнных желез, криоглобулинемию, спленомегалию, персистирующую системную активность и лимфаденопатию, так как частота данного заболевания достаточно высока в этой популяции пациентов

Cutaneous Manifestations of Nutritional Excess: Pathophysiologic Effects of Hyperglycemia and Hyperinsulinemia on the Skin.

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 diabeticorum in a patient: un uncommon etiology of restrictive lung pattern Escleredema diabeticorum en un paciente: una etiología infrecuente de patrón restrictivo pulmonar

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.

Complete remission of scleredema adultorum of Buschke associated with multiple myeloma after bortezomib-based treatment and autologous peripheral blood stem cell transplantation

Complete remission of scleredema adultorum of Buschke associated with multiple myeloma after bortezomib-based treatment and autologous peripheral blood stem cell transplantation

Monoclonal Gammopathy-Associated Scleredema Adultorum of Buschke in a Patient with Diabetes Mellitus Successfully Treated with Intravenous Immunoglobulin and Narrow-Band Ultraviolet B Phototherapy: A Case Report.

Monoclonal Gammopathy-Associated Scleredema Adultorum of Buschke in a Patient with Diabetes Mellitus Successfully Treated with Intravenous Immunoglobulin and Narrow-Band Ultraviolet B Phototherapy: A Case Report.

Diabetes-associated dermatological manifestations in primary care and their association with vascular complications.

Although type 2 diabetes mellitus (DM) is a global public health problem, the diabetes-associated dermatological (non-infectious) manifestations (DADM) remain poorly understood and under-diagnosed. We aimed to evaluate the prevalence of 7 known DADM in a primary care setting, and their association macro/microvascular complications. Cross-sectionnal study included patients consulting in general practice for DM-follow up, from November 2016 to January 2017. Patients aged <18years old or consulting for other reason than DM follow up were excluded. Each patient were screened for diabetic dermopathy (DD), Huntley's papules (HP), necrobiosis lipoidica diabeticorum (NL), acanthosis nigricans (AN), cheiroarthropathy (CA, or stiff hand syndrom), scleredema adultorum of Buschke (SB) and bullosis diabeticorum (BD). 213 diabetic patients were included over a period of 3months. We found a prevalence of 17.8% (38 patients) for DD, 8.5% (18) for HP, 2.8% (6) for NL, 2.3% (5) for AN, 1.9% (4) for CA, 1.4% (3) for SB and 1.4% (3) for BD. DADM seems to be a risk factor for vascular complications (OR 1.97, p≤ 0.001). Association with vascular involvement was stronger with DD and macroangiopathy (OR 1.86, p≤ 0.001), and with NL and microangiopathy (OR 9.7, p ≤ 0.001). In primary care, DM-associated dermatological manifestations present similar prevalence rates to a tertiary care setting, based on litterature. Complete dermatological examination of diabetic patients is essential and could lead to a better overall management of the pathology, as diabetic cutaneous manifestations appear as a sign of vascular involvement.

Buschke Scleredema: A new case in diabetic patient

Buschke Scleredema: A new case in diabetic patient

Sclerosing skin diseases

Sclerosing skin manifestations are more a symptom than a diagnosis and must therefore be meticulously clarified. Systemic scleroderma as amultiorgan 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.

A Case of Scleredema Diabeticorum: Could Pentoxifylline Be a Therapeutic Option?

Background: Scleredema diabeticorum is a dermatosis characterized by hardening of skin over the upper back. It is commonly seen in poor-controlled diabetic patients. Therapeutic options are limited. Psoralen and ultraviolet A therapy (PUVA), methotrexate, and topical steroids have been used with some success. Pentoxifylline is a xanthine derivative used mainly in peripheral vascular diseases. It also has antifibrotic effects due to its collagen synthesis inhibition action. Case Presentation: In this paper, a trial of intralesional pentoxifylline treatment and magnetic resonance images of a male patient with scleredema diabeticorum are presented. Some subjective symptoms of the patient improved after this treatment, but, the thickness of the lesion did not change. Conclusion: Pentoxifylline, probably via the oral route, could still be thought in such patients unresponsive to conventional approaches due to its antifibrotic activity and high safety profile.

Scleredema adultorum : Clinical presentation, diagnostic workup, differential diagnosis, treatment option

Scleredema adultorum (SA) is arare 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 achronic 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.

Cutaneous manifestations of diabetes mellitus: a clinical study

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Diabetes mellitus is a leading cause of morbidity with adverse effects on various systems including skin. Cutaneous manifestations seen in diabetes aid in its diagnosis and some of them may also correlate with diabetic control, duration and other complications. This study was undertaken to estimate burden of skin disorders among diabetics in a tertiary care centre of Uttarakhand.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; The study was conducted at Himalayan Institute of Medical Sciences, Dehradun, Uttarakhand, India, over a period of one year from January 2016 to December 2016. Three hundred and seventy seven patients irrespective of age and sex were included in the study and those with gestational diabetes were excluded. Patients were examined for mucocutaneous manifestations and evaluated. Structured case reporting form was used to generate data.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; Out of 377 patients studies, 114 (30.2%) were in the age group of 51-60 years. Male to female ratio was 1.44:1. Mucocutaneous manifestations were present in 278/377 (73.7%) patients. Common cutaneous manifestations were cutaneous infections [fungal infections (n=106/377; 28.1%), bacterial infections (n=33/377; 8.8%)], pruritus (n=47/377; 12.5%), xerosis (n=39/377; 10.3%), diabetic dermopathy (n=36/377; 9.5%) and diabetic ulcers (n=24/377; 6.4%). Uncommon manifestations were necrobiosis lipoidica diabeticorum, alopecia areata, scleredema diabeticorum and granuloma annulare. Cutaneous reactions due to treatment of diabetes were noted in 4 (1%) patients.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; As prevalence of cutaneous manifestations is high in diabetic patients, knowledge of these will help in a timely and comprehensive management of both diabetes and dermatoses.&lt;/p&gt;