Ultrasonographic Findings of Scleredema Adultorum of Buschke Involving the Posterior Neck

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.

A Case of Widespread Scleredema Treated with Cyclosporine and Steroid / 대한피부과학회지

No abstract available.

Scleredema associated with Sjögren’s syndrome

Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Further investigation led to the diagnosis of primary Sjögren’s syndrome. The association between scleredema and autoimmune disorders has been rarely seen. To our knowledge, there are no other reports describing the association between primary Sjögren’s syndrome and scleredema adultorum of Buschke.

Scleredema of Buschke Following Streptococcal Infection

No abstract available.

Scleredema diabeticorum: A case report

Scleredema is an uncommon condition of unknown etiology that is characterized by dermal mucinosis and mild sclerosis. It is a symmetrical, diffuse, non-pitting induration of the skin commonly associated with an antecedent febrile illness, diabetes mellitus or paraproteinemia.This is the case of an obese, middle-aged adult female with type 2 diabetes mellitus on oral hypoglycemic medication, who presented with an ill-defined, diffuse erythema and non-pitting induration of the skin on the posterior neck and upper back. It has been estimated that as many as 2.5%-14% of diabetic patients have scleredema. This subset of patients may be under-reported as a consequence of subtle onset and under- recognition. Even lithe prognosis of scleredemadiabeticorum is usually benign, it is important that this condition is recognized since it may have systemic involvement that can lead to complications.

Escleredema de Buschke asociado a infección estreptocócica

El escleredema de Buschke, o adultorum, es una enfermedad rara del tejido conjuntivo, de causa desconocida, caracterizada por una induración súbita, simétrica y difusa de la piel, de difícil tratamiento. Se presenta un paciente pediátrico con escleredema de Buschke secundario a amigdalitis estreptocócica, manejado satisfactoriamente con fototerapia con rayos ultravioleta B de banda estrecha y penicilina oral.

A Case of Scleredema Associated with Dermatomyositis

Dermatomyositis (DM) is rare systemic inflammatory disease with typical skin manifestations and muscular involvement. Various skin lesions can accompany this disease, such as Gottron's sign, Heliotrope rash, mechanic's hands, V sign and shawl sign. Scleredema is a very rare skin manifestation in DM. We report a case of DM in a 63-year-old woman, who had scleredema on her thighs. A diagnosis of DM was established by clinical manifestation, elevated muscle enzyme levels, electromyogram measures, and muscle biopsy findings. She was successfully treated with the immunosuppressants methotrexate, cyclosporine, and steroids (low dose).

A Case of Scleredema Mimicking Systemic Sclerosis with Hypoalbuminemia Induced by Malabsorption in Alcoholic Chronic Pancreatitis

Scleroderma pathogenesis is the accumulation of extracellular matrix proteins and is a relatively rare connective tissue disorder characterized by skin fibrosis, obliterative vasculopathy, and distinct autoimmune abnormalities. However, many other clinical conditions known collectively as the scleroderma-like syndrome present with substantial skin fibrosis and may be confused with scleroderma, sometimes leading to an incorrect diagnosis. Due to this, early and correct diagnosis is very important to for appropriate treatment available for scleroderma-like syndrome. We report a rare case of scleredema mimicking systemic sclerosis with hypoalbuminemia induced by malabsorption in alcoholic chronic pancreatitis. In this case, the patient's skin sclerosis and joint contracture dramatically improved after high dose steroid theraphy.

Escleredema de Buschke associado ao diabetes melito tipo 2: relato de caso e revisão da literatura / Scleredema of Buschke associated with diabetes mellitus type 2: case report and review of the literature

Escleredema de Buschke (EB) é doença rara de tecido conjuntivo, caracterizada por endurecimento não depressível da pele. De etiologia desconhecida, pode estar associada com infecções bacterianas ou virais, alterações hematológicas e diabetes melito (DM). Em metade dos casos publicados de EB, há associação com DM. As características mais comuns nesses pacientes são: adultos do sexo masculino, DM de longa duração, mal controle metabólico e presença de complicações específicas do DM. O exame histopatológico revela aumento da espessura da derme, com fibras de colágeno alargadas e separadas por espaços não corados, os quais cor-respondem a depósitos de mucopolissacarídeos. Consequências clínicas incluem: diminuição da motilidade dos ombros e comprometimento da função respiratória. Diversos tratamentos são propostos na literatura, porém com resultados inconstantes. Relata-se caso de EB em paciente diabético tipo 2.

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.

Coexistence of Scleredema and Acanthosis Nigricans in a Patient with Diabetes Mellitus / 대한피부과학회지

Scleredema and acanthosis nigricans are two distinct dermatological disorders that have been reported in association with insulin resistance. Few reported cases have shown an association between scleredema and acanthosis nigricans. A 53-year-old obese woman with diabetes mellitus presented with a 4-year history of an indurated plaque on the nape and upper mid-back. She also presented with a 20-year history of hyperpigmented, velvety plaques on the neck and both axillae. We report a diabetic patient who developed scleredema and acanthosis nigricans.

Clinical and pathologic characteristics of patients with scleredema / 中国医学科学院学报

<p><b>OBJECTIVE</b>To investigate the clinical and pathologic characteristics of patients with scleredema.</p><p><b>METHOD</b>The clinical and pathologic data of 67 outpatients with scleredema who were admitted to PUMC Hospital from 1982 to 2008 were retrospectively analyzed.</p><p><b>RESULTS</b>Neck and upper back lesions were detected in all patients, but no hand or foot involvement was noted. Among 54 patients who received serum immunoglobulin examination, 19 (35.2%) showed abnormal serum immunoglobulin results. Among 67 patients, 22 (32.8%) had concomitant diabetes mellitus. Alcian blue staining was performed in 35 patients, among whom 23 (65.7%) had positive results and 12 (34.3%) had negative results.</p><p><b>CONCLUSIONS</b>Scleredema may have systemic involvements in addition to skin lesions. Patients with scleredema also tends to have concomitant diabetes mellitus. Alcian blue staining is not sufficient to differentiate scleredema and scleroderma.</p>

Scleredema of Buschke: a rare post-streptococcal complication.

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.

Escleredema diabético: presentación de un caso y revisión de la literatura / Diabetic escleredema: presentation of a case and revision of literature

El escleredema es una entidad infrecuente de etiología desconocida, que se caracteriza por el engrosamiento de la piel del cuello, los hombros y la espalda. Generalmente se observa en pacientes diabéticos. Presentamos el caso de un varón de 74 años, diabético tipo II, que cursa internación en la unidad de cuidados intensivos por un accidente cerebro-vascular y neumonía grave. Al examen dermatológico presentaba una placa de escleredema en cuello, hombros y parte alta del dorso, sobre la que asentaba una úlcera provocada por el decúbito obligado.

[Scleredema of Buschke. A case report]

Scleredema of Buschke is a rare disease whose aetiology remains unknown. It is characterized by symetric skin induration of the upper part of the body which usually resolve spontaneously. It can be associated with extra-cutaneous symptoms. We report a new case in a 55 year old women

A Comparison of the Fraction of Collagen and Elastic Fibers by Image Analysis in Scleredema / 대한피부과학회지

BACKGROUND: Scleredema is a rare connective tissue disorder, characterized by hard and thick skin. It is well known that collagen synthesis is increased in scleredema skin, but there has been no study on the change of elastic fibers. OBJECTIVE: The purpose of this study was to compare the changes of collagen and elastic fibers in scleredema and normal skin. METHODS: We retrospectively evaluated the biopsy specimens of 10 patients diagnosed with scleredema. The control group was selected by matching each scleredema case with same site and similar ages from the biopsy specimens which showed normal dermis. A computerized image analysis system was used to measure the fraction of collagen and elastic fibers and calculate the ratio of elastic fibers to collagen fibers in each group. RESULTS: The skin of scleredema was found to have a significant increase in the percentage of collagen fibers in the dermis compared to the control group. On the other hand, the percentage of elastic fibers was significantly decreased in the scleredema group (p<0.05). CONCLUSION: In contrast to scleroderma which shows an increase in dermal elastic tissue and collagen fibers, we concluded that there is a decrease of elastic fibers in scleredema and that the pathogenesis and molecular involvement are different in the two diseases. we also found that the computerized image analysis system was a useful tool in the measurement of connective tissue components.

Scleredema of Buschke in pediatric age group

Two cases of scleredema of Buschke are described, which occurred in pediatric age group - an uncommon occurrence after febrile illness. Both cases were self-limiting. Characteristic features are described

Widespread Scleredema Accompanied with a Monoclonal Gammopathy in a Patient with Advanced Ankylosing Spondylitis

Scleredema is a rare cutaneous mucinosis characterized by chronic diffuse induration of the skin, and it is occasionally associated with a monoclonal gammopathy (MG). Ankylosing spondylitis (AS) is noted to be another, chronic systemic inflammatory disorder of the axial skeleton that may accompany the MG. However, patients with scleredema and AS accompanied with a MG have not been reported in the literature. We here report a 40-yr-old man with scleredema and advanced AS accompanied with a MG of IgA-kappa protein. Widespread, long-standing scleredema has been developed over 10 yrs after the initial manifestation of AS. It is uncertain whether the coexistence of scleredema and AS is more than coincidental.