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PubMedDate not supplied

[Buschke Scleredema, case report].

Buschke Scleredema is a rare connective tissue disorder of unknown aetiology, characterized by thickening of the dermis whose characteristics may mainly to mime systemic sclerosis, eosinophilic fasciitis and cutaneous amyloidosis. Scleredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. Scleredema can be classified into three clinical groups; each has a different history, course, and prognosis. Each one of these share reduction in chest articular movements and limitation of limbs movements. The skin histology is characterised by thickened dermis and increased spaces between large collagen bundles due to increased deposition of mucopolysaccharide in the dermis. Differential diagnosis can be made considering the typical clinical features and the histologic peculiarity. No therapy has been found effective. The authors describe a case of Buschke Scleredema successfully treated by steroids and colchicine. Clinical evaluation of skin induration and thickness as well as ultrasonography were performed at baseline and after treatment.

Adrenal Cortex Hormones↗
PubMed2005

Infliximab-induced scleredema in a patient with rheumatoid arthritis.

A 52-year-old patient with rheumatoid arthritis (RA) developed scleredema-like skin induration after treatment with the tumor necrosis factor alpha (TNFalpha) blocking agent, infliximab. Skin induration occurred within a few weeks of initiation of infliximab, resolved with discontinuation of the drug, and recurred with rechallenge with the drug, implicating infliximab as the offending agent. Laboratory evaluation revealed a high titer of human antichimeric antibodies (HACA). The skin induration improved within a few weeks of discontinuation of infliximab and did not recur with the use of etanercept. Scleredema has been reported in association with bacterial and viral infections, diabetes mellitus, and monoclonal gammopathies. Infliximab use should be added to the list of potential associations with scleredema. This effect appears possibly specific to infliximab and may be related to the development of HACA because it did not occur with the use of etanercept in this patient. In addition, there appears to be a complex relationship between TNFalpha and tumor growth factor beta (TGF-beta), a cytokine which promotes collagen synthesis and deposition. TNFalpha blockade with infliximab may affect TNFalpha-TGF-beta interactions and may be implicated in the development of scleredema in this case.

Antibodies, Monoclonal↗
PubMed1981

Monoclonal gammopathy in scleredema. Observations in three cases.

A monoclonal gammopathy was observed in three patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. Deposition of monoclonal immunoglobulins in the skin was not detected by direct immunofluorescence microscopy. In contrast to scleromyxedema (lichen myxedematosus), from which scleredema can be distinguished clinically and histologically, the monoclonal immunoglobulins in two cases were of IgG2-kappa and IgG3-kappa type. Only one of the three patients had IgG1-lambda paraproteinemia, which is frequently seen in scleromyxedema. Our findings suggest that diffuse scleredema may be characterized by paraproteinemia but that the possible role of monoclonal immunoglobulins in the pathogenesis of this disease has yet to be resolved.

Adult↗
PubMed2004

Radiotherapy of benign diseases-scleredema adultorum Buschke.

BACKGROUND: Scleredema adultorum Buschke is a rare disorder characterized by thickening of the dermis of the neck, head and upper trunk. Its etiology is unknown, but there may be a preceding history of infection and there is a known association with diabetes mellitus. Women are more frequently affected. Usually, the disease is self-limiting but some patients show progressive disease. In these cases therapeutic options are poor, with only case reports and small series supporting their use. CASE REPORT: A 58-year-old patient with a scleredema of the neck and upper trunk is described, who was treated twice within 6 months by electron-beam radiation therapy. After the second course his symptoms improved significantly. A review of the literature of radiation treatment of this disease is given. CONCLUSION: . Regardless of the possible mechanisms in pathogenesis and treatment of scleredema adultorum Buschke, the application of ionizing radiation is an important, effective and well-tolerated therapy option in the treatment of severe cases and may candidate as the first-line treatment of this disease.

Humans↗
PubMed2005

Scleredema adultorum of Buschke presenting as periorbital edema: a diagnostic challenge.

Scleredema adultorum is a rare sclerotic disorder characterized by diffuse swelling and nonpitting induration of the skin. Its occurrence has been documented in association with infections, diabetes mellitus, paraproteinemia, multiple myeloma, and monoclonal gammopathy. We report an unusual case of a 48-year-old man with an asymptomatic bilateral eyelid edema of sudden onset. During a period of 6 months, the condition slowly progressed to extensive nonpitting edematous swelling restricted to the periorbital sites. The presumptive diagnosis of scleredema adultorum was confirmed by the presence of typical histologic findings. This case is unique in that the periorbital swelling remained as the sole clinical manifestation of scleredema during the 5-year follow-up and was complicated with partial vision blockage.

Collagen↗
PubMedDate not supplied

[Scleredema of Buschke associated with diabetes mellitus. Study of four cases].

Scleredema of Buschke is characterized by a thickening of the dermis with mucin deposits among the collagen fibers, which manifests as a hardening of the skin, predominantly on the upper trunk. It has been associated with long-standing, poorly controlled diabetes mellitus, monoclonal gammopathies and streptococcus infections. We studied patients with a clinical diagnosis of scleredema confirmed by means of a histopathological study, and associated with diabetes mellitus. We reviewed associated diseases, clinical and histopathological characteristics, evolution and response to treatment. Scleredema is characterized by hardening of the skin, affecting the upper trunk. A large proportion of cases are associated with long-standing, poorly controlled diabetes mellitus, with little response to treatment.

Adult↗
PubMed2006

Scleredema adultorum of Buschke: an under recognized skin complication of diabetes.

Scleredema of Buschke or scleredema diabetorum is a skin complication of diabetes with deposits of collagen and aminoglycans in the dermis. This disease characterized by thickening and hardening of the skin, is usually localized in nape, back and shoulder areas. Consequences could be a decrease in motility of the shoulders and an impairment of respiratory function. Other possible complications are sleep apnoea syndrome and monoclonal gammapathy. Type 1 or type 2 diabetes may be associated with scleredema of Buschke in more than 50% of cases. Diabetes-related risk factors are long duration of the disease, presence of microangiopathy, overweight and need of insulin. Various specific treatments proposed in the literature are poorly validated. In most severe cases, radiation therapy may be useful.

Adult↗
PubMedDate not supplied

Scleredema adultorum associated with hyperkeratosis.

Scleredema adultorum is a rare skin disorder reported to occur usually following streptococcal infections. It has characteristic clinical and histopathologic findings; however, associated hyperkeratosis is not known. We report a 6-year-old girl with scleredema adultorum and hyperkeratosis. The patient's disease had a benign course. To our knowledge, this patient is the first reported with scleredema adultorum associated with hyperkeratosis.

Anti-Bacterial Agents↗
PubMedDate not supplied

Scleredema adultorum.

Scleredema adultorum is a rare connective tissue disorder reported usually following streptococcal infection, influenza, measles, and mumps. It has been reported occasionally following trauma and tuberculous lymphadenitis. This is a report of scleredema adultorum developing after chicken pox in an eight-year-old male child. The diagnosis was established by characteristic picture on skin biopsy using special stain. The patient had a benign course and a spontaneous recovery in two weeks. The case has been reported as the first case of scleredema adultorum developing after chicken pox.

Chickenpox↗
PubMed2002

Scleredema diabeticorum--a case report.

A 41 year male, diabetic on treatment presented with persistent erythema and. "taut" skin over neck and back since 1 year. The lesions showed symmetric and gradual progression. There was no history of prior sore throat. On examination effected skin was erythematous, woody hard and unpinchable. Scleredema diabeticorum (diutinum), although sharing clinical and historical features with Scleredema adultorum has no prodromal infection, is more extensive and affected individuals are characteristically obese, middle aged diabetics who often have accompanying microangiopathies and macroangiopathies. Recognition of scleredema by the physician has prognostic and therapeutic implications in the management of the coexisting diabetes.

Adult↗
PubMed2004

Scleredema of Buschke: remission with factor XIII treatment.

We describe the case of a 55-year-old man with scleredema of Buschke of the torso complicated by insulin-dependent diabetes mellitus. Due to (i) the patient's poor general health status, (ii) the similarity between scleroderma and scleredema of Buschke, and (iii) the well known efficacy of factor XIII infusions in scleroderma, we attempted an intravenous treatment with factor XIII. This therapy resulted in marked increase of movements and in softening of the skin, together with ultrasonographic and histopathological improvements. In conclusion, to the best of our knowledge, this is the first case in which factor XIII has been successfully used for the treatment of scleredema of Buschke.

Diabetes Mellitus, Type 1↗
PubMed1989

[Clinical analysis of 60 cases of scleredema].

Clinical analysis of 60 cases of scleredema is reported with emphasis on clinical manifestations, microcirculatory changes, histopathological and ultrastructural observations. It is noted that the prognosis of scleredema may not always be good, and severe cases may lose their working capacity or even die. Attention should be paid to proper treatment of scleredema and long term follow-up is indicated to observe its course, outcome and pattern of evolution.

Adolescent↗
PubMed1987

Scleredema adultorum associated with a monoclonal gammopathy and generalized hyperpigmentation.

Scleredema associated with a monoclonal gammopathy and generalized skin pigmentation is described in a 56-year-old man with hyperlipoproteinemia and cardiovascular disease. The patient had IgG-lambda paraproteinemia, without any evidence of multiple myeloma or immunoglobulin deposition in affected skin. Ultrastructural studies of pigmented lesional skin showed increased transfer of melanosomes to basal keratinocytes and dermal melanophages containing complex melanosomes. In addition, cytoplasmic, electron-opaque lipid droplets were seen in approximately every third keratinocyte or melanocyte, while only an occasional dermal cell contained lipid droplets. The hyperpigmentation appeared to be directly related to the scleredema, while the lipid deposition in skin was a likely consequence of the hyperlipoproteinemia. The findings further support the contention that paraproteinemia and hyperpigmentation may, in some patients, be associated features of scleredema adultorum.

Histocytochemistry↗
PubMed1986

Scleredema in an infant.

A 3-month-old infant with scleredema had associated cytomegalovirus pneumonia with a rapidly fatal course. Because differentiation of scleredema from scleredema neonatorum, subcutaneous fat necrosis, and scleroderma may be difficult, histologic diagnosis is important. Cetylpyridinium chloride was used successfully as a fixative to demonstrate acid mucopolysaccharides histologically.

Biopsy↗
PubMed1984

Scleredema and monoclonal gammopathy: report of two cases.

Two patients had scleredema with monoclonal gammopathy, one of whom was considered to have smoldering multiple myeloma. In one patient, the scleredema cleared without treatment, while in the other, the scleredema and the monoclonal gammopathy persisted unchanged.

Adult↗
PubMed1995

Improvement of scleredema associated with IgA multiple myeloma after chemotherapy.

The presence of a monoclonal gammopathy in patients with chronic scleredema is a recently described, unusual association of unknown significance. There have been very occasional reports of multiple myeloma in such patients. We now report a patient with long-standing scleredema who developed an IgA-kappa multiple myeloma. Once chemotherapy for the myeloproliferative disorder was instituted, scleredema improved significantly, suggesting a pathogenetic link between the two conditions.

Aged↗
PubMed2003

Scleredema adultorum of Buschke treated with radiation.

BACKGROUND: Scleredema adultorum of Buschke is an uncommon skin condition that may accompany diabetes mellitus. Current therapeutic options are poor, with only case reports and small series supporting their use. OBSERVATION: We describe 3 patients with severe restrictive scleredema associated with type 2 diabetes mellitus who responded to radiation therapy after other treatments had failed. Angle of shoulder abduction was used as an objective measurement of improvement, while the patients rated several subjective areas, including arm range of motion, ease of performing daily tasks, ease of breathing, and neck thickening. Objective improvement tended to plateau over time but remained well above baseline. The duration of subjective improvement was variable, lasting from a few months to the end of the observation time of 2 1/2 years. CONCLUSION: Though not consistently durable in its effect, radiation therapy appears to be a viable therapeutic option in extreme cases of this difficult-to-treat disease.

Follow-Up Studies↗
PubMedDate not supplied

The ultrasonic evaluation of skin thickness in scleredema.

The skin thickness in a group of patients with scleredema was measured using ultrasonic techniques and then compared with a group of normal individuals. The mean dermal thickness in scleredema was 7.7 mm, compared to 3.3 mm in the normal control group. Ultrasonic evaluation of skin thickness may be of value in following the natural history or response to therapy of certain diseases that affect dermal thickness.

Humans↗