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At least 55 records · Page 3Linked to original sources
PubMed2000

Scleroderma-like syndrome in a child: eosinophilic fasciitis or scleredema adultorum?

UNLABELLED: A 6-year-old female patient presented with a rapidly progressive scleroderma-like syndrome involving almost the entire integument. Initially clinical patterns and histopathological data of both eosinophilic fasciitis and scleredema adultorum were present. The course of the disease remained unusual for both conditions but finally argued in favour of the diagnosis of eosinophilic fasciitis. CONCLUSION: Eosinophilic fasciitis and scleredema adultorum might be subtypes of one disease entity.

Child, Preschool↗
PubMed1992

Scleredema and smoldering myeloma.

A 46-year-old white man had classic findings of scleredema; subsequently, a monoclonal immunoglobulin G lambda light chain was detected in his serum. Findings of a bone marrow biopsy specimen revealed that he had an increased percentage of plasma cells, some of which were atypical. A diagnosis of scleredema and smoldering myeloma was made.

Humans↗
PubMed1984

Scleredema: a review of thirty-three cases.

A review of thirty-three cases of scleredema, with particular reference to clinical and histologic findings, revealed that the disease was often of insidious onset and the course was usually prolonged. A preceding respiratory tract infection was uncommon and usually did not indicate a short course. The patients were divided into two groups according to the presence or absence of diabetes. Diabetes, when present, was typically the late-onset, insulin-dependent type and difficult to control. In many of these patients, the onset of scleredema was so subtle that it went unnoticed by the patient until pointed out by an examining physician.

Adolescent↗
PubMed1998

In vivo study of skin mechanical properties in scleredema of Buschke.

A non-invasive, in vivo suction device was used to investigate the mechanical properties of the skin in a patient with scleredema of Buschke. Clinical scoring of skin induration and measurements of skin elasticity were performed over 9 anatomic regions on admission and after 3 (on discharge), 17 and 28 months. Immediate distension, final distension and immediate retraction were significantly decreased, while the viscoelastic to elastic ratio was significantly increased in the patient as compared to the healthy controls. Delayed distension and biological elasticity were preserved. Low values of skin distensibility correlated with a severe skin induration (p < 0.001). The changes were more expressive with the 8 mm-diameter measuring probe than the 2 mm-diameter probe. The method applied can be used for objective and quantitative assessment of skin involvement in scleredema of Buschke.

Elasticity↗
PubMed1994

Electron-beam therapy in scleredema adultorum with associated monoclonal hypergammaglobulinaemia.

We report a case of scleredema adultorum (Buschke's disease) associated with an IgA kappa monoclonal hypergammaglobulinaemia. A significant improvement in the skin was obtained with electron-beam therapy. Scleredema would appear to be linked to monoclonal hypergammaglobulinaemia, but the relationship between the skin disorder and the immunoglobulin abnormality remains to be elucidated.

Adolescent↗
PubMed2005

Successful UVA1 phototherapy in a patient with scleredema adultorum.

Scleredema adultorum is a rare connective tissue disorder of unknown cause. Both bath-PUVA and cream-PUVA therapy were reported to be effective. We describe a patient with scleredema adultorum who showed a striking clinical improvement with a medium-dose UVA1 phototherapy (single dose, 50 J/cm(2); 35 treatments).

Humans↗
PubMed2004

UVA-1 as a treatment for scleredema.

Scleredema is a rare disease that is difficult to treat. Many therapies have been tried with varied and somewhat inconsistent results. Here we report two cases of scleredema successfully treated with low-dose UVA-1.

Adult↗
PubMed1996

Increased collagen propeptides in the skin of a scleredema patient but no change in re-epithelialisation rate.

Scleredema is a rare disease, affecting the skin connective tissue with increased amounts of collagen and glycosaminoglycans. In the present study, the collagen synthesis and re-epithelialisation rate were measured from a 64-year-old male patient, who rapidly developed extensive tightening of the skin, without any underlying disease. The skin was thickened at several sites when measured with ultrasound, and the histology revealed accumulation of glycosaminoglycans and collagen bundles. The collagen synthesis rate was measured from suction blisters induced on two different sites of the skin before the treatment and three times later up to 6 months after the treatment with a systemic steroid was started. The aminoterminal propeptide of type I collagen (PINP) was increased manifold in the affected skin when compared with the controls, indicating active collagen deposition in vivo. Systemic steroid medication with high doses (over 20 mg/d) decreased both the type I and the type III collagen propeptide levels. The time schedule of the decreases in the propeptides in the thickened, affected skin and in the clinically normal-looking skin varied, and especially in the thickened skin in the abdomen the decrease in PINP was noted only after 3 months of prednisolone therapy. When the prednisolone dose was only 10 mg daily, the propeptides were again up-regulated, perhaps reflecting the natural course of the disease. The re-epithelialisation rates at two different sites of the patient were similar to those in the controls, suggesting that even massive fibrosis with active deposition of collagen does not alter the basal rate of re-epithelialisation in the skin. In conclusion, collagen synthesis is markedly elevated in scleredema, leading to fibrosis of the skin. A recently developed method utilizing assays of collagen propeptides from suction blister fluid allows monitoring of the collagen synthesis and detection of changes in the collagen synthesis during the treatment of fibrotic disorders.

Abdomen↗
PubMed2006

An unusual finding in scleredema.

Scleredema of Buschke is a rare disorder in which areas of induration appear in the skin, commonly after a remote infection and sometimes with diabetes and para-proteinemias. We report an unusual finding in an adult patient of long standing classical scleredema who developed chronic clustered papules/pseudovesicular lesions with koebnerization after a bacterial infection of the upper back.

Diabetes Mellitus, Type 1↗
PubMed2005

Buschke's scleredema and concomitant diseases: report of five cases and literature review.

Scleredema adultorum or Buschke's scleredema is a rare disorder that belongs to the group of mucinoses. Diffuse, sudden swelling, hardening and induration of the skin can occur in children and younger women as well as in older men. The dermis is thickened because of the increased collagen glycosylation, like that in diabetic stiff skin syndrome. The face is most often involved. In older persons the trunk is usually first involved. There is relative sparing of the extremities, with no distal or Raynaud's phenomenon. Eosinophilic fasciitis, scleromyxedema, associated gammopathy, and other forms of edema and mucin deposition must be excluded on differential diagnosis. Antibiotics, sometimes high doses of intravenous penicillin, systemic corticosteroids, systemic PUVA and PUVA bath therapy seem most promising for the management of the disorder. Our patients were aged 27, 60, 64, 69 and 72 years, with typical skin lesions, thus that term "adultorum" does not appear to fit well.

Adult↗
PubMed1990

Acute scleredema with fatal outcome.

Death due to scleredema is rare. We report a patient with scleredema, which had an acute onset and rapid progression, leading to death within a month. Such an acute course terminating in fatality has not been described earlier.

Acute Disease↗
PubMed1983

Scleredema adultorum of Buschke: a clinical, pathologic, and cell culture study of two patients.

Two patients with scleredema of Buschke are presented. The second patient developed scleredema after a febrile drug reaction. Biopsies of both involved and uninvolved skin were obtained for histologic examination and cell culture studies. Immunofluorescent studies of the biopsy specimens revealed staining with IgG, IgM and C3 at the dermal-epidermal junction in the involved skin of the first patient. This has not been previously reported. Cell culture studies revealed that fibroblasts from the involved skin produced more glycosaminoglycans (GAG) than uninvolved skin and most of the GAG produced was hyaluronic acid. Possible pathogenic mechanisms for this unusual condition are discussed.

Biopsy↗
PubMed1983

[Buschke's scleredema and monoclonal dysglobulinemia: apropos of 3 cases].

A monoclonal gammopathy was observed in 3 adult patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. The monoclonal immunoglobulin was in every case of the IgG-kappa type. Bence Jones proteinuria was noted in 1 case (kappa). Deposition of monoclonal IgG-kappa in the skin was not detected by immunofluorescence microscopy. These findings and previous reports of 5 cases suggest that diffuse scleredema may be frequently associated with paraproteinemia, but the role of monoclonal immunoglobulins in the pathogenesis of the disease remains to be clarified.

Adolescent↗
PubMedDate not supplied

[Buschke's scleredema and carditis: a clinical case].

An 11-year old girl who presented non-pitting edema of the face and neck, shoulder pain and a gallop rhythm about 10 days after a febrile episode with sore throat is described. The mask-like facial appearance and limited movement of the neck led to suspect scleredema which may be accompanied by carditis. Skin and muscle biopsy findings confirmed the diagnosis. Cardiac involvement included a gallop rhythm persisting for 6 months, ECG changes consisting in transitory T-wave inversion in leads II and V2 - V4, a transitory pericardial function rub and hepatomegaly due to right-sided failure which responded to digitalization. In this case the presence of a gallop rhythm and elevated ASLO titer led to an initial diagnosis of acute rheumatic fever; dermatomysitis and sclerodermia are also to be considered in the differential diagnosis of scleredema of Buschke. In our patient the edema resolved completely within 12 months, the gallop rhythm within 6 months and the ECG became normal after 8 months.

Arrhythmias, Cardiac↗
PubMed1982

Scleredema adultorum of Buschke with upper esophageal involvement.

A 55-yr-old white female with biopsy proven scleredema adultorum was evaluated for progressive dysphagia. Esophageal motility studies revealed persistently high upper esophageal pressures without response to deglutition, and minimal peristalsis in the upper body of the esophagus. A cine-esophagram revealed massive aspiration of barium into the nasopharynx and lungs, concommitant with stenosis in the upper esophagus. The patient died presumptively from aspiration. This patient exemplifies a potentially lethal complication of scleredema adultorum i.e., upper esophageal infiltration with resultant aspiration.

Deglutition Disorders↗
PubMed1980

[Scleredema "adultorum" (Buschke) (author's transl)].

In relation to a new case of scleredema "adultorum" (Buschke) observed by authors in a two year-old girl, they compare characteristics of this type of cutaneous induration with those of sclerodermia. Scleredema affects the neck region, face and upper thorax, and very rarely it reaches the hands, meanwhile in sclerodermia the hands and face, and some other possible cutaneous region and viscera are commonly affected. The former regresses in six months to two years, or rarely later; the second is progressive and permanent. Histologically, dermal collagen in increased in both but in the former the fibers are not condensed and hyalinized, as they are in the second.

Child, Preschool↗