ISSN 0001-5555 ActaDV Volume 100 2020 Theme issue ADVANCES IN DERMATOLOGY AND VENEREOLOGY A Non-profit International Journal for Interdisciplinary Skin Research, Clinical and Experimental Dermatology and Sexually Transmitted Diseases Frontiers in Dermatology and Venereology - A series of theme issues in relation to the 100-year anniversary of ActaDV Official Journal of - European Society for Dermatology and Psychiatry Affiliated with - The International Forum for the Study of Itch Immediate Open Access Acta Dermato-Venereologica www.medicaljournals.se/adv ACTA DERMATO-VENEREOLOGICA The journal was founded in 1920 by Professor Johan Almkvist. Since 1969 ownership has been vested in the Society for Publication of Acta Dermato-Venereologica, a non-profit organization. Since 2006 the journal is published online, independently without a commercial publisher. (For further information please see the journal’s website https://www. medicaljournals.se/acta) ActaDV is a journal for clinical and experimental research in the field of dermatology and venereology and publishes high- quality papers in English dealing with new observations on basic dermatological and venereological research, as well as clinical investigations. Each volume also features a number of review articles in special areas, as well as Correspondence to the Editor to stimulate debate. New books are also reviewed. The journal has rapid publication times. Editor-in-Chief: Olle Larkö, MD, PhD, Gothenburg Former Editors: Johan Almkvist 1920–1935 Deputy Editors: Sven Hellerström 1935–1969 Anette Bygum, MD, PhD, Odense Nils Thyresson 1969–1987 Magnus Lindberg, MD, PhD, Örebro Lennart Juhlin 1987–1999 Elisabet Nylander, MD, PhD, Umeå Anders Vahlquist 1999–2017 Kaisa Tasanen-Määttä, MD, PhD, Oulu Artur Schmidtchen 2018–2019 Section Editors: Tasuku Akiyama, Miami (Neurodermatology and Itch - Experimental) Annamari Ranki, Helsinki (Cutaneous lymphoma) Nicole Basset-Seguin, Paris (Skin cancer) Artur Schmidtchen, Lund (Wound healing and Innate immunity) Veronique Bataille, London (Melanoma, Naevi, Photobiology) Matthias Schmuth, Innsbruck (Genodermatoses and Keratinizing disorders, Josip Car, Singapore (Health Services Research and e-Health) Ichthyosis and Retinoids) Brigitte Dréno, Nantes (Acne and Rosacea) Lone Skov, Hellerup (Psoriasis) Regina Fölster-Holst, Kiel (Paediatric dermatology, Atopy and Parasitoses) Enikö Sonkoly, Stockholm (Psoriasis and related disorders) Jürg Hafner, Zürich (Skin cancer, Skin tumours, and Melanoma) Jacek Szepietowski, Wrocław (Psychodermatology) Jürgen Harder, Kiel (Cutaneous innate defense, Skin microbe interactions) Elke Weisshaar, Heidelberg (Itch and Neurodermatology) Roderick Hay, London (Cutaneous Infections) Margitta Worm, Berlin (Atopic dermatitis and Immunology) Kristian Kofoed, Copenhagen (STD and Microbiology) Claus Zachariae, Hellerup (Contact dermatitis, Acute dermatology) Veli-Matti Kähäri, Turku (Skin cancer) Magnus Ågren, Copenhagen (Wound healing & Extracellular matrix) Dennis Linder, Graz/Padua (Psychoderm., Dermato -epidemiology, e-Health) Advisory Board: Masashi Akiyama, Nagoya Rudolf Happle, Freiburg Lisa Naysmith, Edinburgh Mona Ståhle, Stockholm Wilma Bergman, Leiden Lars Iversen, Aarhus Jonathan Rees, Edinburgh Sonja Ständer, Münster Tilo Biedermann, Munich Peter van de Kerkhof, Nijmegen Jean Revuz, Paris Jouni Uitto, Philadelphia Magnus Bruze, Malmö Irene Leigh, Dundee Johannes Ring, Munich Shyam Verma, Vadodara Earl Carstens, Davis John McGrath, London Matthias Ringkamp, Baltimore Gil Yosipovitch, Miami Thomas Diepgen, Heidelberg Maja Mockenhaupt, Freiburg Inger Rosdahl, Linköping Giovanna Zambruno, Rome Charlotta Enerbäck, Linköping Dedee Murrell, Sydney Thomas Ruzicka, Munich Christos C. Zouboulis, Dessau Kilian Eyerich, Stockholm All correspondence concerning manuscripts, editorial matters and subscription should be addressed to: Acta Dermato-Venereologica S:t Johannesgatan 22, SE-753 12 Uppsala, Sweden Editorial Manager, Mrs Agneta Andersson Editorial Assistant: Ms Anna-Maria Andersson Please send an E-mail Please send an E-mail Information to authors: Acta Dermato-Venereologica publishes papers/reports on scientific investigations in the field of dermatology and venereology, as well as reviews. Case reports and good preliminary clinical trials or experimental investigations are usually published as Short Communications. However, if such papers are of great news value they could still be published as full articles. Special contributions such as extensive feature articles and proceedings may be published as supplements to the journal. For detailed instructions to authors see https://www.medicaljournals.se/acta/instructions-to-author. Publication information: Everything is Open Access and no subscription fee. For publication fees: see https://www.medicaljournals.se/acta/ instructions-to-author. Indexed in: See https://www.medicaljournals.se/acta/about/adv. Frontiers in Dermatology and Venereology DV Centenary theme issues in Volume 100 of Acta Dermato-Venereologica a t An overview c A Current issue Blistering skin disorders Theme editor: Kaisa Tasanen • Skin Fragility: Perspectives on Evidence-based Therapies, L. Bruckner-Tuderman • Collagen XVII Processing and Blistering Skin Diseases, W. Nishie • Drug Development in Pemphigoid Diseases, K. Bieber, R.J. Ludwig • Current Concepts of Dermatitis Herpetiformis, T. Salmi, K. Hervonen • Bullous Drug Reactions, M. Mockenhaupt enereologica V Forthcoming issues ermato- Genodermatoses D Theme editors: Anette Bygum and Matthias Schmuth cta • Ichthyosis: A Road Model for Skin Research, A. Vahlquist, H Törmä A • An Early Description of a “Human Mosaic” Involving the Skin: A Story from 1945, R. Happle • Dental Manifestations of Ehlers-Danlos Syndromes – A Systematic Review, I. Kapferer-Seebacher, D. Schnabl, J. Zschocke, F. M. Pope • Diagnosis and Management of Inherited Palmoplantar Keratodermas, B.R. Thomas, E.A. O’Toole • Molecular Genetics of Keratinization Disorders - What’s New, • E. Denayer, E. Legius V • Genetics of Inherited Ichthyoses and Related Diseases, J. Fischer, E. Bourrat • H. Bonnekoh, M. Butze, T. Kallinich, N. D Kambe, G. Kokolakis, K. Krause a t c Skin malignancies A Theme editors: Veronique Bataille and Nicole Basset Seguin Content details TBA Atopic dermatitis Theme editors: Magnus Lindberg and Carl-Fredrik Wahlgren Content details TBA Cutaneous and genital infections Theme editors: Roderick Hay and Kristian Kofoed Content details TBA dvances in dermatology and venereology A Previous issues Itch and pruritic disorders DV Theme editors: Elke Weisshaar, Tasuku Akiyama and Jacek Szepietowski cta • The Challenge of Basic Itch Research, E. Carstens, T. Follansbee, M.I. Carstens • Mechanisms and Management of Itch in Dry Skin, C. Sagita Moniaga, M. Tominaga, K. Takamori A • Non-dermatological Challenges of Chronic Itch, A.E. Kremer, T. Mettang, E. Weisshaar • Itch and Psyche: Bilateral Associations, R. Reszke, J.C. Szepietowski • A New Generation of Treatments for Itch, E. Fowler, G. Yosipovitch • Challenges in Clinical Research and Care in Pruritus, M.P. Pereira, C. Zeidler, M. Storck, K. Agelopoulos, W.D. Philipp-Dormston, A.G.S. Zink, S. Ständer Psoriasis Theme editors: Lone Skov and Enikö Sonkoly • Psoriasis and Genetics, N. Dand, S. Mahil, F. Capon, C.H. Smith, M.A. Simpson and J. Barker enereologica • The Woronoff Ring in Psoriasis and the Mechanisms of Postinflammatory Hypopigmentation, J. Prinz V • Psoriasis and Treatment: Past, Present and Future Aspects, C. Reid, C.E.M. Griffiths • Psoriasis and Co-morbidity, M. Amin, E.B. Lee, T-F. Tsai, J.J. Wu • Pustular Psoriasis: the Dawn of a New Era, H. Bachelez ermato- D cta A DV cta A dvances in dermatology and venereology A Theme issue: Blistering skin disorders DV cta A enereologica V BLISTERING SKIN DISORDERS ermato- D Theme Editor: cta A Kaisa Tasanen DV cta A TABLE OF CONTENTS Skin Fragility: Perspectives on Evidence-based Therapies, L. Bruckner-Tuderman 94–101 Collagen XVII Processing and Blistering Skin Diseases, W. Nishie 102–107 Drug Development in Pemphigoid Diseases, K. Bieber, R.J. Ludwig 108–114 Current Concepts of Dermatitis Herpetiformis, T. Salmi, K. Hervonen 115–121 Bullous Drug Reactions, M. Mockenhaupt 122–134 dvances in dermatology and venereology A Centenary theme section: BLISTERING SKIN DISORDERS REVIEW ARTICLE DV Skin Fragility: Perspectives on Evidence­based Therapies cta Leena BRUCKNER-TUDERMAN Department of Dermatology, Medical Faculty and Medical Center – University of Freiburg, Freiburg, Germany A The term skin fragility disorders describes a group of SIGNIFICANCE conditions in which the structural integrity of the skin is compromised and its resistance to external shear The term skin fragility describes skin that blisters and forces diminished. Skin fragility can have different cau- breaks easily upon mild friction or trauma. Skin fragility ses, ranging from genetic variations to inflammatory can have many causes, ranging from genetic variants to a or physical phenomena. The genetic skin fragility dis- compromised immune system, infections or adverse drug orders, collectively called epidermolysis bullosa, serve reactions. Studies of genetic skin fragility disorders, such enereologica as a paradigm for the study of causes and mechanisms as epidermolysis bullosa, have provided better understan- V of skin fragility. Recent biomedical research has revea- ding of their causes and mechanisms. At least 20 genes led substantial genetic heterogeneity of the epidermo- may be involved in