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Giornale Italiano di Dermatologia e Venereologia 2018 October;153(5):599-608

DOI: 10.23736/S0392-0488.18.06073-X


language: English

Italian Guidelines in Pemphigus - adapted from the European Dermatology Forum (EDF) and European Academy of Dermatology and Venerology (EADV)

Claudio FELICIANI 1 , Emanuele COZZANI 2, Angelo V. MARZANO 3, Marzia CAPRONI 4, Giovanni DI ZENZO 5, Piergiacomo CALZAVARA-PINTON 6, on behalf of “Cutaneous Immunology” group of SIDeMaST 

1 Dermatologic Clinic, University of Parma, Parma, Italy; 2 Dermatologic Clinic, University of Genoa, Genoa, Italy; 3 Unit of Dermatology, Department of Medical-Surgical Physiopathology and Transplantation, IRCCS Ca’ Granda Foundation, Maggiore Policlinico Hospital, University of Milan, Milan, Italy; 4 SOS Rare Dermatological Diseases, Unit of Dermatology, Piero Palagi Hospital, Usl Toscana Centro, University of Florence, Florence, Italy; 5 Molecular and Cell Biology Laboratory, Istituto Dermopatico dell’Immacolata (IDI), IRCCS, Rome, Italy; 6 Dermatologic Clinic, University of Brescia, Brescia, Italy

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 in collaboration with the European Academy of Dermatology and Venereology. It summarizes evidence-based and expert-based recommendations (S2 level).

KEY WORDS: Pemphigus - Guidelines - Skin diseases, vesiculobullous

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