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G Ital Dermatol Venereol. 2018 May 31. doi: 10.23736/S0392-0488.18.06073-X. [Epub ahead of print]

Italian guidelines in pemphigus. Adapted from the European Dermatology Forum (EDF) and European Academy of Dermatology And Venerology (EADV).

Author information

1
Clinica Dermatologica, Università di Parma, Parma, Italy - claudio.feliciani@unipr.it.
2
Clinica Dermatologica, Università di Genova, Genoa, Italy.
3
U.O.C. Dermatologia, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
4
SOS Malattie Rare Dermatologiche, U.O. Dermatologia I, P.O. Piero Palagi, Usl Toscana Centro, Università di Firenze, Florence, Italy.
5
Molecular and Cell Biology Laboratory, Istituto Dermopatico dell'Immacolata (IDI)-IRCCS, Rome, Italy.
6
Clinica Dermatologica, Università di Brescia, Brescia, Italy.

Abstract

Pemphigus is a life threatening autoimmune epidermal blistering disease involving skin and mucous membranes. Pemphigus usually affects middle age men and women involving oral mucosa first and then spreading on the skin. It is caused by the presence of autoantibodies (IgG and less frequently by IgA) directed against desmogleins, and/or other glycoproteins that plays a critical role in cell-cell attachment. Upon a predisposing genetic background, different agents have been shown to act as triggers for the pathogenesis of pemphigus. This guideline for the diagnosis and treatment of pemphigus has been developed by an Italian group of experts taking in account the Italian legislation and local pharmacological governance. Guidelines are adapted from the original article under the guidance of the European Dermatology Forum (EDF) in collaboration with the European Academy of Dermatology and Venereology (EADV). It summarises evidence-based and expert-based recommendations (S2 level).

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