ChloroquineV1, Main, Exploration, bibRecord, 002A83

Scleromyxedema

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Scleromyxedema

Auteurs : Annette M. Dinneen [États-Unis] ; Charles H. Dicken [États-Unis]

Source :

Journal of the American Academy of Dermatology [ 0190-9622 ] ; 1995.

RBID : ISTEX:19000C80F6E5F1E7BC84627DD84E8AD0BE9913E0

English descriptors

Teeft :
- Abnormal paraprotein, Acute leukemia, Alkylating agents, American academy, Arch dermatol, Biclonal peak, Carpal tunnel, Chemotherapeutic agents, Collagen bundles, Congestive heart failure, Dermatol, Dermatology, Dermatology july, Dermatology volume, Dicken, Diffuse sclerosis, Dinneen, Dramatic response, Extracutaneous manifestations, Fibroblast proliferation, Generalized papular eruption, Hematologic malignancies, Histologic evidence, Inflammatory myopathy, Lichen, Lichen myxedematosus, Mayo clinic, Melphalan, Mucin deposition, Mucinous material, Multiple myeloma, Myeloma, Myopathy, Myxedematosus, Natural history, Papillary dermis, Papular, Papular mucinosis, Paraprotein, Scleromyxedema, Scleromyxedema myopathy, Septic complications, Short term, Significant toxicity, Skin disease, Systemic manifestations, Treatment group.

Abstract

Abstract: Background: Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and that is associated with significant morbidity and mortality. Objective: Our purpose was to study the natural history of the disease and its response to therapy with alkylating agents. Methods: A clinicopathologic review of 26 patients with scleromyxedema was performed, and the extracutaneous findings and response to therapy with alkylating agents were noted. Results: Extracutaneous manifestations, most often gastrointestinal, were present in 20 of 26 patients. An abnormal paraprotein was found in 23 of 26 patients, most commonly IgG-λ (18 patients). Melphalan was used as therapy for 17 patients. The disease proved fatal in 10 of the treated patients. Conclusion: The overall prognosis in scleromyxedema is poor. Therapy is difficult. Although alkylating agents may prove beneficial in the short term, significant toxicity of these agents is apparent with long-term use.

Url:

https://api.istex.fr/ark:/67375/6H6-XC7ZW53L-D/fulltext.pdf

DOI: 10.1016/0190-9622(95)90007-1

Affiliations:

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Le document en format XML

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<series><title level="j">Journal of the American Academy of Dermatology</title>
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<term>Alkylating agents</term>
<term>American academy</term>
<term>Arch dermatol</term>
<term>Biclonal peak</term>
<term>Carpal tunnel</term>
<term>Chemotherapeutic agents</term>
<term>Collagen bundles</term>
<term>Congestive heart failure</term>
<term>Dermatol</term>
<term>Dermatology</term>
<term>Dermatology july</term>
<term>Dermatology volume</term>
<term>Dicken</term>
<term>Diffuse sclerosis</term>
<term>Dinneen</term>
<term>Dramatic response</term>
<term>Extracutaneous manifestations</term>
<term>Fibroblast proliferation</term>
<term>Generalized papular eruption</term>
<term>Hematologic malignancies</term>
<term>Histologic evidence</term>
<term>Inflammatory myopathy</term>
<term>Lichen</term>
<term>Lichen myxedematosus</term>
<term>Mayo clinic</term>
<term>Melphalan</term>
<term>Mucin deposition</term>
<term>Mucinous material</term>
<term>Multiple myeloma</term>
<term>Myeloma</term>
<term>Myopathy</term>
<term>Myxedematosus</term>
<term>Natural history</term>
<term>Papillary dermis</term>
<term>Papular</term>
<term>Papular mucinosis</term>
<term>Paraprotein</term>
<term>Scleromyxedema</term>
<term>Scleromyxedema myopathy</term>
<term>Septic complications</term>
<term>Short term</term>
<term>Significant toxicity</term>
<term>Skin disease</term>
<term>Systemic manifestations</term>
<term>Treatment group</term>
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<front><div type="abstract" xml:lang="en">Abstract: Background: Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and that is associated with significant morbidity and mortality. Objective: Our purpose was to study the natural history of the disease and its response to therapy with alkylating agents. Methods: A clinicopathologic review of 26 patients with scleromyxedema was performed, and the extracutaneous findings and response to therapy with alkylating agents were noted. Results: Extracutaneous manifestations, most often gastrointestinal, were present in 20 of 26 patients. An abnormal paraprotein was found in 23 of 26 patients, most commonly IgG-λ (18 patients). Melphalan was used as therapy for 17 patients. The disease proved fatal in 10 of the treated patients. Conclusion: The overall prognosis in scleromyxedema is poor. Therapy is difficult. Although alkylating agents may prove beneficial in the short term, significant toxicity of these agents is apparent with long-term use.</div>
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<name sortKey="Dicken, Charles H" sort="Dicken, Charles H" uniqKey="Dicken C" first="Charles H" last="Dicken">Charles H. Dicken</name>
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Scleromyxedema

Scleromyxedema

Source :

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri