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Serration pattern analysis for differentiating epidermolysis bullosa acquisita from other pemphigoid diseases - 14/03/18

Doi : 10.1016/j.jaad.2017.11.029 
Joost M. Meijer, MD a, Ingeborg Atefi b, Gilles F.H. Diercks, MD, PhD a, Artem Vorobyev, MD b, Janny Zuiderveen a, Hillegonda J. Meijer a, Hendri H. Pas, PhD a, Detlef Zillikens, MD, PhD b, Enno Schmidt, MD, PhD b, c, Marcel F. Jonkman, MD, PhD a,
a Department of Dermatology, Center for Blistering Diseases, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands 
b Department of Dermatology, University of Lübeck, Lübeck, Germany 
c Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany 

Correspondence to: Marcel F. Jonkman, MD, PhD, Department of Dermatology, University Medical Center Groningen, Hanzeplein 1, Groningen 9700 RB, The Netherlands.Department of DermatologyUniversity Medical Center GroningenHanzeplein 1Groningen9700 RBThe Netherlands

Abstract

Background

Direct immunofluorescence (DIF) microscopy of a skin biopsy specimen is the reference standard for the diagnosis of pemphigoid diseases (PDs). Serration pattern analysis enables the differentiation of epidermolysis bullosa acquisita (EBA) from other PDs using DIF microscopy alone. However, practice gaps need to be addressed in order to implement this technique in the routine diagnostic procedure.

Objective

We sought to determine and optimize the technical requirements for serration pattern analysis of DIF microscopy and determine interrater conformity of serration pattern analysis.

Methods

We compared serration pattern analysis of routine DIF microscopy from laboratories in Groningen, The Netherlands and Lübeck, Germany with 4 blinded observers. Skin biopsy specimens from 20 patients with EBA and other PDs were exchanged and analyzed. Various factors were evaluated, including section thickness, transport medium, and biopsy specimen processing.

Results

The interrater conformity of our 4 observers was 95.7%. Recognition of serration patterns was comparable in samples transported in saline and in Michel's medium and with section thicknesses of 4, 6, and 8 μm.

Limitations

Limitations include our small sample size and the availability of 20 samples that were compared retrospectively.

Conclusion

DIF serration pattern analysis is not restricted by variation in laboratory procedures, transport medium, or experience of observers. This learnable technique can be implemented as a routine diagnostic method as an extension of DIF microscopy for subtyping PD.

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Graphical abstract




Il testo completo di questo articolo è disponibile in PDF.

Key words : autoimmune bullous disease, basement membrane, dermatopathology, diagnostic technique, direct immunofluorescence microscopy, epidermolysis bullosa acquisita, pemphigoid

Abbreviations used : DIF, EBA, EBMZ, PD


Mappa


 Funding sources: None.
 Dr Zillikens has served on the advisory boards of, been a consultant for, and received either speaker honoraria or grants and research funding from Almirall, Biogen, Biotest, Dompe, Euroimmun, Fresenius, Janssen, Miltenyi, Roche, and UCB. Dr Jonkman has shares in Philae Pharmaceuticals, has been a consultant for Roche/Genentech, and has received a grant from Castle Creek Pharma. The other authors have no conflicts of interest to disclosed.


© 2017  American Academy of Dermatology, Inc.. Pubblicato da Elsevier Masson SAS. Tutti i diritti riservati.
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