Scleredema diabeticorum is a distinct cutaneous condition peculiar to diabetic individuals and ought not to be confused with scleredema of buschke or scleroderma. Request pdf on feb 1, 2019, olga simoguerrero and others published escleredema diabeticorum. We report the case of a 41yearold man with type 1 diabetes who presented with scleredema diabeticorum. Scleredema diabeticorum with superimposed cellulitis and. Electronic publications 80540 freely accessible full text publications plus those not yet available due to embargo. Scleredema diabeticorum is a rare complication of diabetes, characterized by diffuse induration of the skin over the neck, shoulders, and upper trunk and arms, progressively leading to limited upper body motility and impaired respiratory function. Drei typen des sa werden entsprechend ihrer assoziation zu vorbestehenden bzw. Scleredema of buschke is a connective tissue disease of unknown etiology, characterized by an excess of dermal acid mucopolysaccharides. Scleredema adultorum of buschke is a rare, benign disease that results in symmetrical induration and thickening of the skin due to increased collagen and mucin deposition in the dermis. Etiology is unknown, and histopathological findings include thickened dermis with abundant mucin deposition among large collagen bundles. Pdf scleredema diabeticorum in a patient with type 2. Scleredema diabeticorum is generally associated with noninsulindependent diabetes mellitus. Association with other complications of diabetes is not certain and treatment is difficult, though improvement is possible with optimal glycaemic control. Psoralen and ultraviolet a therapy puva, methotrexate, and topical steroids have been used with some success.
Acid mucopolysaccharide staining in scleredema cole. Scleredema diabeticorum of buschke confined to the thighs. Scleredema adultorum of buschke is a rare fibromucinous connective tissue disease belonging to the group of primary mucinoses and sclerodermalike disorders. Scleredema of buschke is a connective tissue disease of unk nown etiology. Scleredema diabeticorum is an infrequent connective tissue disorder described for the first time by buschke in the year 1900. Scleredema, is a rare, selflimiting skin condition defined by progressive thickening and hardening of the skin, usually on the areas of the upper back, neck, shoulders and face. Scleredema adultorum, a connective tissue disorder of unknown aetiology, is characterized by a thickening of the reticular dermis in the upper back of the body that may decrease the. Severe scleredema diabeticorum and insulin resistance. Pdf scleredema diabeticorum in a patient with type 2 diabetes. Read scleredema diabeticorum of buschke confined to the thighs, british journal of dermatology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Pdf scleredema diabeticorum a case report researchgate.
Scleredema is a rare condition of unknown pathogenesis. Scleredema is an uncommon fibromucinous connectivetissue disease. Scleredema diabeticorum is a syndrome characterized by a marked increase in dermal thickness on the posterior back and upper neck in middleaged, overweight, poorly controlled type 2 diabetic subjects fig. Single stains were positive in only half the tissues. This item appears in the following collections faculty of medical sciences 68674. It is a poorly known recognised complication of diabetes. Nuchal fibroma associated with scleredema, diabetes.
Scleredema diabeticorum is rare and is a cause of significant morbidity in diabetes. We report the unique case of a patient with no underlying comorbidities who presented with asymptomatic scleredema. Up to 1965, only two hundred twentythree cases of scleredema had been reported worldwide. In a prospective study of 484 diabetic outpatients we found. Signs and symptoms of this condition include hardening and thickening of the skin which may restrict movement. Scleredema diabeticorum in a patient with type 2 diabetes. We present a case of scleredema with a leonine facies in a 56yearold man with a history of poorly controlled diabetes mellitus. Limited options are available to treat patients with scleredema diabeticorum.
Scleredema genetic and rare diseases information center. Microscopic examination shows a thickened dermis with large swollen collagen bundles, particularly type 1 collagen, separated by. Glycation of skin collagen and skin hypoxia due to microangiopathy may be key factors. The patient initially presented with erythematous, edematous papules and plaques on the face, neck, and upper back.
We present a case of scleredema diabeticorum sd in a patient with diabetic neuropathy and restrictive respiratory disease with unusual skin lesion distribution. Treatment of scleredema diabeticorum with tamoxifen the. Scleroedema should not be confused with scleroderma, in which the skin is fibrotic morphoea and systemic sclerosis. The third group, known as scleredema diabeticorum sd, is a chronic form of scleredema associated with severe, often complicated, diabetes mellitus dm. Scleredema diabeticorum often has a subtle onset, persistent involvement and is refractory to therapies. The aetiology of scleredema diabeticorum is unclear. It is commonly seen in poorcontrolled diabetic patients.
It is not recognized as being related to digital sclerosis, and we found no correlation by ultrasound measurements between back skin and hand skin thickness. It is also known as scleredema adultorum, scleredema adultorum of buschke, scleredema diabeticorum, and scleredema diabeticorum of buschke. It is worthy to note that diabetes is observed in half of the cases of scleredema 17 rho yw, suhr kb, lee jh, park jk. Aidsrelated insulin resistance and lipodystrophy syndrome are a newly emerging entities in hivinfected patients.
Scleredema diabeticorum with unusual presentation and fatal outcome francisco j ruiz miyares 1, renju kuriakose 1, dirk t deleu 1, naghi abd elwahad 2, hassan alhail 1 1 department of neurology, hamad general hospital, doha, qatar 2 department of radiology, hamad general hospital, doha, qatar. Scleredema diabeticorum in a patient with type 2 diabetes mellitus. Observation we describe 3 patients with severe restrictive scleredema associated with type 2 diabetes mellitus who responded to radiation therapy after other treatments. A new appraisal of dermatologic manifestations of diabetes. Histologically, there is a remarkable deposition of mucin along the deep dermis layers. A case of idiopathic scleredema adultorum of buschke. It usually occurs in patients with poorly controlled or insulinresistant diabetes mellitus. A clinical observation of scleredema adultorum and its relationship to diabetes. Scleredema is a form of cutaneous mucinosis caused by an increased accumulation of collagen and mucin in the dermis.
It is characterized by diffused, nonpitting swelling and induration of the skin. It is characterized clinically by woody induration and hardening of the skin that results from excessive mucin deposition between thickened collagen bundles in the skins dermis. Pentoxifylline is a xanthine derivative used mainly in peripheral vascular diseases. A relationship to diabetes mellitus was established in 1970 and the term scleredema diabeticorum is often used in this setting. A case report alessandra gandolfi, andrea pontara, gaetano di terlizzi, nathalie rizzo, roberto nicoletti, marina scavini, gabriella galimberti, emanuele bosi. Scleredema in a patient with aidsrelated lipodystrophy. Scleredema adultorum of buschke treated with radiation.
Scleredema diabeticorum is a rare cutaneous manifestation of diabetes mellitus. It is considered an adult disease, but can also occur in children. Scleredema diabeticorum with unusual presentation and. Type 1 scleredema is referred to as adultorum of buschke since the postinfectious form of scleredema was the one, first described by buschke himself. Nuchal fibroma associated with scleredema, diabetes mellitus and organic solvent exposure nuchal fibroma associated with scleredema, diabetes mellitus and organic solvent exposure banney, leith a. Microscopic examination shows a thickened dermis with large swollen collagen bundles, particularly type 1 collagen, separated by ground substance and wide clear spaces. Treatment of scleredema diabeticorum with tamoxifen. Scleredema diabeticorum has a reported a prevalence of 2. Current therapeutic options are poor, with only case reports and small series supporting their use. It is a disease of long standing diabetes, associated with obesity. Sd is characterized by an insidious onset of skin thickening, occurring diffusely over the posterior neck and upper back and occasionally extending to the deltoid and lumbar regions figure. It is classically divided into three types, based on its association with postinfection usually streptococcal, monoclonal gammopathy and diabetes mellitus scleredema diabeticorum.
Scleredema diabeticorum is an uncommon skin condition. Of the 17 scleredema patients diagnosed by us in the last 15 yr, 16 have had type ii diabetes mellitus. Scleredema adultorum, a connective tissue disorder of unknown aetiology, is characterized by a thickening of the reticular dermis in. Successful treatment of scleredema diabeticorum by. Scleredema diabeticorum a case report sciencedirect. Scleroedema american spelling scleredema is a type of cutaneous mucinosis of unknown cause. It develops in the skin on the upper part of the back, shoulders, and neck. It can be classified in a diabetic accounting for 2550% of cases and a nondiabetic type, that includes an idiopathic, a postinfective, a monoclonal gammopathyassociated type and one described in association with some anecdotal.
Signs and symptoms of this condition include hardening and thickening of. Scleredema, an uncommon condition with unknown pathophysiologic characteristics, is characterized by nonpitting induration of the skin with occasional erythema. Clinically it may be preceded by an acute febrile illness, usually infectious. Scleredema of buschke associated with diabetes mellitus type 2. We present a case of an obese male with poorly controlled. Scleredema, originally described by buschke in 19021, is a rare sclerodermatosis of unknown etiology, characterized by nonpitting induration of the skin.
Onset of scleredema diabeticorum is insidious, and a progressive, nonpitting induration of the skin involving the posterior neck, back, and shoulders, and less commonly arms, is often seen. Scleredema is an uncommon, but likely underrecognized, fibromucinous connectivetissue disease. Identifying rheumatology mimics sharon l kolasinski md facp facr professor of clinical medicine division of rheumatology perelman school of medicine university of pennsylvania february 2016. Scleredema of buschke sb is a rare disorder of connective tissue, characterized by hardening of the skin. This entity should be distinguished from other primary autoimmune blistering disorders and from simple mechanobullous lesions. Scleredema diabeticorum in a patient with the normal range. Scleredema diabeticorum sd can be considered as a major dermatological complication of diabetes mellitus dm. Answer scleredema diabeticorum causes a thickening of the skin on the back of the neck and upper back. Scleredema is a form of cutaneous mucinosis, a diverse group of rare skin conditions that are characterized by an accumulation of mucin a jellylike complex carbohydrate substance in the skin. It is characterized clinically by woody induration and hardening of the skin as the result of excessive mucin deposition between thickened collagen bundles in the skins dermis. Scleredema diabeticorum is characterized by a dramatic increase in the thickness of the skin of the posterior neck and upper back. Improvement in clinical symptoms of scleredema diabeticorum by frequencymodulated electromagnetic neural stimulation.
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