Treatment of recalcitrant scleromyxedema with thalidomide in 3 patients

Sansbury, Julia C.; Cocuroccia, Barbara; Jorizzo, Joseph L.; Gubinelli, Emanuela; Gisondi, Paolo; Girolomoni, Giampiero

doi:10.1016/j.jaad.2004.01.058

Cited by 69 publications

(50 citation statements)

References 20 publications

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“…This treatment can be given alone or in combination with IV Ig. 11,12 Some reports have shown response to melphalan plus autologous hematopoietic stem cell transplant and bortezomib plus dexamethasone.…”

Section: Discussionmentioning

confidence: 99%

Scleromyxedema: A Case Report with Cutis-Laxa like Features

Molgó¹

2018

MOJGG

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Scleromyxedema is an uncommon disease that corresponds to the generalized form of lichen myxedematosus. It is clinically characterized by generalized papules with skin infiltration. Histopathology reveals a triad including mucin deposition, fibrosis and irregularly arranged fibroblastic proliferation. This disease presents multiple extracutaneous manifestations and is associated with other diseases, especially paraproteinemia. We present the case of a 75 year-old woman, who presented a scleromyxedema with cutis-laxa like features. These cutaneous changes are infrequently seen and have been recently described in the context of scleromyxedema.

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Section: Discussionmentioning

confidence: 99%

Scleromyxedema: A Case Report with Cutis-Laxa like Features

Molgó¹

2018

MOJGG

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“…Scleromyxedema is a rare systemic mucinosis that is most commonly seen in middle-aged patients with an underlying IgG lambda gammopathy. It is estimated that less than 10% of patients with scleromyxedema will progress to overt multiple myeloma [13,14]. The remaining 90% of patients develop progressive infiltration of their internal organs associated with significant morbidity and mortality [4,12,14].…”

Section: To the Editormentioning

confidence: 99%

Scleromyxedema and dermato–neuro syndrome in a patient with multiple myeloma effectively treated with dexamethasone and bortezomib

et al. 2011

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“…Historically, melphalan therapy has been the treatment of choice for this condition with multiple reports of benefit, but significant toxicity appears frequent (61)(62)(63)(64). Case reports cite variable improvement with other immunosuppressants including cyclophosphamide and cyclosporine (65)(66)(67)(68). There are multiple cases describing some benefit using thalidomide, although there remains a legitimate concern for the development of disabling peripheral neuropathy (68)(69)(70)(71)(72).…”

Section: Treatment and Prognosismentioning

confidence: 99%

“…Case reports cite variable improvement with other immunosuppressants including cyclophosphamide and cyclosporine (65)(66)(67)(68). There are multiple cases describing some benefit using thalidomide, although there remains a legitimate concern for the development of disabling peripheral neuropathy (68)(69)(70)(71)(72). More recent data note benefit of autologous stem cell transplantation in recalcitrant cases (53,(73)(74)(75).…”

Section: Treatment and Prognosismentioning

confidence: 99%

Scleroderma-like Fibrosing Disorders

Boin¹,

Hummers²

2008

Rheumatic Disease Clinics of North America

111

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SynopsisMany conditions presenting with clinical hard skin and tissue fibrosis can be confused with systemic sclerosis (scleroderma). These disorders have very diverse etiologies and often an unclear pathogenetic mechanism. Distinct clinical characteristics, skin histology and disease associations may allow to distinguish these conditions from scleroderma and from each other. A prompt diagnosis is important to spare the patients from ineffective treatments and inadequate management. In this review, we highlighted nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy), eosinophilic fasciitis (Shulman's syndrome), scleromyxedema and scleredema. These are often detected in the primary care setting and referred to rheumatologists for further evaluation. Rheumatologists must be able to promptly recognize them to provide valuable prognostic information and appropriate treatment options for affected patients.

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Treatment of recalcitrant scleromyxedema with thalidomide in 3 patients

Cited by 69 publications

References 20 publications

Scleromyxedema: A Case Report with Cutis-Laxa like Features

Scleromyxedema: A Case Report with Cutis-Laxa like Features

Scleromyxedema and dermato–neuro syndrome in a patient with multiple myeloma effectively treated with dexamethasone and bortezomib

Scleroderma-like Fibrosing Disorders

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