| Scleromyxedema: response to high-dose intravenous immunoglobulin (hdIVIg). | |
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MedLine Citation:
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PMID: 10901735 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We report 2 patients with scleromyxedema, both associated with IgG-lambda paraproteinemia, who were treated with high-dose intravenous immunoglobulin (hdIVIg) 2g/kg per month. The response to treatment was assessed using an objective skin scoring system initially established for patients with scleroderma. This system grades the overall severity of the induration and the reduction in mobility of the skin. Both patients initially had a dramatic response to treatment which was sustained in one patient. The first patient, a 30-year-old black man, showed a reduction in skin scores from 36/60 to 11/60 over a 3-month period, during which time he had 3 infusions of hdIVIg. After an unplanned 2-month break from treatment, severe neuromuscular complications developed. These improved initially with more frequent infusions of hdIVIg but oral corticosteroids were required to treat worsening myopathy. Unfortunately, the initial response to hdIVIg has not been sustained and his skin scores at 1 year returned to baseline. The second patient, a 60-year-old white man, showed a similarly dramatic reduction in skin scores from 36/60 to 15/60 over a 3-month period after having received only 2 infusions of hdIVIg. There has been sustained improvement after 10 months of therapy and the interval between hdIVIg infusions has been increased to 10 weeks without deterioration. HdIVIg may be an effective treatment for some patients with scleromyxedema, a rare condition with few effective treatments and a poor prognosis. |
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Authors:
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R K Lister; S Jolles; S Whittaker; C Black; I Forgacs; M Cramp; M Potter; M H Rustin |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Journal of the American Academy of Dermatology Volume: 43 ISSN: 0190-9622 ISO Abbreviation: J. Am. Acad. Dermatol. Publication Date: 2000 Aug |
Date Detail:
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Created Date: 2000-08-21 Completed Date: 2000-08-21 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 7907132 Medline TA: J Am Acad Dermatol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 403-8 Citation Subset: IM |
Affiliation:
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Department of Dermatology, Royal Free Hospital, London, UK. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Dose-Response Relationship, Drug Drug Administration Schedule Humans Immunization, Passive* Immunoglobulin lambda-Chains / blood Lichen Sclerosus et Atrophicus / diagnosis, immunology, pathology, therapy* Male Middle Aged Paraproteinemias / diagnosis, immunology, pathology, therapy* Scleroderma, Localized / diagnosis, immunology, pathology, therapy* Skin / pathology Treatment Outcome |
| Chemical | |
Reg. No./Substance:
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0/Immunoglobulin lambda-Chains |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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