Autoimmune Bullous Diseases: Text and Review

215 Pages·2016·15.091 MB·English

by  Marcel F. Jonkman (eds.)

Marcel F. Jonkman
Editor
Autoimmune
Bullous Diseases
Text and Review
123
Autoimmune Bullous Diseases

Marcel F. Jonkman
Editor
Autoimmune Bullous
Diseases
Text and Review
Editor
Marcel F. Jonkman
Center for Blistering Diseases
University Medical Center Groningen
Groningen
The Netherlands
ISBN 978-3-319-23753-4 ISBN 978-3-319-23754-1 (eBook)
DOI 10.1007/978-3-319-23754-1
Library of Congress Control Number: 2015956762
Springer Cham Heidelberg New York Dordrecht London
© Springer International Publishing Switzerland 2016
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To my wife Gerrie and my children Lotte, Rutger, and Floris

Pref ace
‘ Knowledge of the history of a disease contributes materially to its under-
standing’. With these words Walter Lever (1909–1992) started his landmark
review in 1953, in which he separated bullous pemphigoid from pemphigus
[1]. The fi rst mentioning of a blistering disease was from François Boissier de
la Croix de Sauvages (1706–1767), who in 1763 introduced the term pemphi-
gus [2]. Since then the number of autoimmune bullous diseases (AIBD) has
expanded to 22 (Fig. 1) . A general physician knows about one by heart: pem-
phigus, but might mix that up with pemphigoid.
AIBD are the most life-threatening infl ammatory diseases of the skin. They
are rare and comprise less than 1 % of the dermatological patients. The subject
AIBD does not cover more than a single page in dermatology study guides for
medical graduates. In general dermatology textbooks for postgraduate resi-
dents, AIBD comprises not more than one chapter. Although textbooks for
autoimmune diseases exist, none are dedicated to AIBD. For many AIBD are

Fig. 1 Tree of autoimmune bullous diseases
vii
viii Preface
confusing diseases that appeal to the medical competence of the specialist.
Here we aim to provide a quick study guide for learning or look-up for the
physician who practices with AIBD patients.
This book is written in direct and simple diction, strait and to the point,
leaving out trivialities and not aiming for absolute completion. Tables and
fi gures illustrate this practical guide to comprehend this ‘diffi cult’ fi eld. At
the end of each chapter, the reader is challenged to check the learned matter
by multiple-choice questions.
The textbook is the effort of the senior staff and PhD’s of the Center for
Blistering Diseases of the University Medical Center Groningen who wrote
this ‘all- Groningen book’. The Center started in 1992 as national expertise
center for blistering diseases in the Netherlands. The Center for Blistering
Diseases has its own diagnostic immunofl uorescence and immune-serologi-
cal laboratory, an electron microscopic facility, and a digital image database
with more than 250,000 dermatological images. The medical photographers
S. Noorman and P. Toonder have taken the excellent photos of patients in this
book. More than 1000 patients with AIBD have visited the Expertise Center
in the last 25 years. The senior authors have gained many years of experience
in clinical and laboratory diagnosis and therapy of AIBD. The ‘Blister
Course’ in Groningen that runs annually since the year 2000 provided much
of the didactic structure used in this book. I wish to thank my dedicated co-
workers for sharing their knowledge to the reader and delivering that in an
unobstructed way. I wish to thank Katarina Ondrekova and Jennifer Schneider
for assisting in obtaining permissions and authorizations. The patients with
AIBD who visited Groningen are thanked for giving us their trust and sharing
their knowledge on symptoms and treatment outcome.
Groningen, The Netherlands Marcel F. Jonkman
References
1. Lever WF. Pemphigus. Medicine. 1953;32:1–123.
2 . Boissier de la Croix de Sauvages, F. Nosologia methodica sistens morborum classes.
Amsterdam: Sumptibus Fratrum de Tournes, 1763. p. 382.
Contents
Part I Immunology and Diagnostic Tests
1 Basic Principles of the Immune System
and Autoimmunity. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
Gilles F. H. Diercks and Philip M. Kluin
2 Dermatological Examination of Bullous Diseases. . . . . . . . . . . 13
Marcel F. Jonkman
3 How to Take a Biopsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
Marcel F. Jonkman and Gilles F. H. Diercks
4 Direct Immunofl uorescence Microscopy . . . . . . . . . . . . . . . . . . 39
Gilles F. H. Diercks and Hendri H. Pas
5 Indirect Immunofl uorescence Microscopy . . . . . . . . . . . . . . . . 47
Gilles F. H. Diercks and Hendri H. Pas
6 Immunoassays . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 57
Hendri H. Pas
Part II Pemphigus
7 Structure of Desmosomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65
Ena S okol
8 Pemphigus Vulgaris. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 71
Gerda van der Wier and Marcel F. Jonkman
9 Pemphigus Foliaceus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 83
Laura de Sena Nogueira Maehara and Marcel F. Jonkman
10 Paraneoplastic Pemphigus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 95
Angelique M. Poot , Gilles F. H. Diercks ,
Hendri H. Pas , and M arcel F. Jonkman
11 IgA Pemphigus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 101
Barbara Horváth and Marcel F. Jonkman
12 Drug-Induced Pemphigus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107
Sylvia H. Kardaun and Laura de Sena Nogueira Maehara
ix