Document Detail


Melkersson-Rosenthal syndrome and acquired C1 inhibitor deficiency.
MedLine Citation:
PMID:  18477847     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Melkersson-Rosenthal syndrome (MRS) is a rare disease whose full-blown form is characterized by orofacial swelling, facial palsy and lingua plicata. OBJECTIVE: To investigate the complement system as well as its role in patients with MRS. METHODS: Seven patients presenting at this hospital between November 2002 and May 2003 and meeting the diagnostic criteria according to Hornstein were evaluated retrospectively. The investigations included clinical signs, an analysis of the complement system including levels of CH50, C3, C4, C1 inhibitor (INH) functions and C1-INH antigen detection. RESULTS: Two female patients showed isolated low levels of functional C1-INH as determined by duplicate tests. Both patients took estrogen-progestin contraceptives. CONCLUSION: Since deficiency in plasma protease C1-INH is known to lead to recurrent angioedema, we hypothesize that low levels of functional C1-INH may have contributed to the orofacial swelling in the 2 patients.
Authors:
F Masson; S Barete; V Frémeaux-Bacchi; H Szpirglas; S Agbo-Godeau; O Chosidow; J C Piette; C Francès
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Publication Detail:
Type:  Journal Article     Date:  2008-05-10
Journal Detail:
Title:  Dermatology (Basel, Switzerland)     Volume:  217     ISSN:  1421-9832     ISO Abbreviation:  Dermatology (Basel)     Publication Date:  2008  
Date Detail:
Created Date:  2008-08-13     Completed Date:  2008-11-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9203244     Medline TA:  Dermatology     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  114-20     Citation Subset:  IM    
Copyright Information:
(c) 2008 S. Karger AG, Basel.
Affiliation:
Department of Dermatology and Allergy, Tenon Hospital, Paris, France.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Biological Markers / analysis*
Combined Modality Therapy
Complement C1 Inactivator Proteins / deficiency*,  immunology
Complement C3 / analysis,  immunology
Complement C4 / analysis,  immunology
Complement Hemolytic Activity Assay
Female
Humans
Male
Melkersson-Rosenthal Syndrome / diagnosis*,  immunology*,  therapy
Middle Aged
Prognosis
Rare Diseases
Retrospective Studies
Risk Assessment
Sampling Studies
Sensitivity and Specificity
Severity of Illness Index
Chemical
Reg. No./Substance:
0/Biological Markers; 0/Complement C1 Inactivator Proteins; 0/Complement C3; 0/Complement C4

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