Document Detail


Scleromyxedema: response to high-dose intravenous immunoglobulin (hdIVIg).
MedLine Citation:
PMID:  10901735     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
R K Lister; S Jolles; S Whittaker; C Black; I Forgacs; M Cramp; M Potter; M H Rustin
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
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:
Created Date:  2000-08-21     Completed Date:  2000-08-21     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7907132     Medline TA:  J Am Acad Dermatol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  403-8     Citation Subset:  IM    
Affiliation:
Department of Dermatology, Royal Free Hospital, London, UK.
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MeSH Terms
Descriptor/Qualifier:
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:
0/Immunoglobulin lambda-Chains

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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