Document Detail

IF serration pattern analysis as a diagnostic criterion in anti-laminin-332 mucous membrane pemphigoid - immunopathological findings and clinical experience in 10 Dutch patients.
MedLine Citation:
PMID:  21692774     Owner:  NLM     Status:  Publisher    
Background:  Anti-laminin-332 mucous membrane pemphigoid (anti-LN-332 MMP) is a chronic subepidermal blistering disease characterized by IgG anti-epidermal basement membrane zone (BMZ) autoantibodies against laminin-332 (LN-332). Patients with anti-LN-332 MMP have an increased relative risk of malignancy. For diagnosis of anti-LN-332 MMP difficult to obtain laboratory techniques are needed. Objectives:  To incorporate direct immunofluorescence (DIF) serration pattern analysis of IgG depositions in the diagnostic criteria of anti-LN-332 MMP. Patients/Methods:  Patients that met our revised inclusion criteria for anti-LN-332 MMP were selected from our biobank over the period 1997-2009. Inclusion criteria were clinical symptoms, DIF serration pattern analysis, indirect immunofluorescence on salt-split skin, and antigen-specificity analysis of the serum including immunoblotting and/or immunoprecipitation and/or ELISA against native LN-332. Results:  Ten patients met the inclusion criteria. A malignancy was found in two patients (20%). In all patients in whom it was performed (n=9), DIF showed linear IgG deposition along the BMZ in an n-serrated pattern. Nine sera reacted by salt-split skin analysis and bound to the dermal side of the split skin. ELISA against native LN-332 was positive in 77.8% of the tested sera. Conclusions:  Anti-LN-332 MMP can clinically resemble other forms of pemphigoid. Although state of the art laboratory diagnostics are necessary for definite diagnosis, the combination of simple DIF serration pattern and IIF salt-split skin analysis will exclude other forms of MMP and epidermolysis bullosa acquisita from the differential diagnosis. Because of the increased risk for malignancy patients should be thoroughly oncologically screened.
J B Terra; H H Pas; M Hertl; F G Dikkers; N Kamminga; M F Jonkman
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-6-21
Journal Detail:
Title:  The British journal of dermatology     Volume:  -     ISSN:  1365-2133     ISO Abbreviation:  -     Publication Date:  2011 Jun 
Date Detail:
Created Date:  2011-6-22     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0004041     Medline TA:  Br J Dermatol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 British Association of Dermatologists.
Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands Department of Dermatology, Philipps University of Marburg, Germany Department of Otorhinolaryngology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands Department of Ophthalmology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
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