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1. Italian guidelines in pemphigus - adapted from the European Dermatology Forum (EDF) and European Academy of Dermatology and Venerology (EADV)

Author

Feliciani C, Cozzani E, Marzano AV, Caproni M, Di Zenzo G, Calzavara-Pinton P, 'Cutaneous Immunology\\' group of SIDeMaST (Alaibac M, Antiga E, Arisi M, Assalve D, Atzori L, Babino G, Balato A, Balato N, Barbagallo T, Belloni Fortina A, Berardesca E, Berti E, Borghi A, Brazzelli V, Brumana MB, Brusati C, Caccavale S, Caldarola G, Campanati A, Caposiena Caro RD, Coati I, Conti A, Costanzo A, Dattola A, De Simone C, Di Cesare A, Di Lernia V, Di Zenzo GM, Fania L, Filosa G, Filotico R, Fusano M, Gerarda Galluccio A, Ganzetti G, Gisondi P, Guarneri F, Hansel K, Javor S, La Placa M, Liberati G, Lora V, Merlo G, Nasca MR, Nigro A, Noal C, Offidani A, Papini M, Parodi A, Patrizi A, Peserico A, Piaserico S, Prignano F, Quaglino P, Rossi M, Satta R, Schena D, Sena P, Simonetti O, Stan TR, Stingeni L, Talamonti M, Tavecchio S, Tognetti L, Vascellaro A, Vassallo C, Venturini M, Zampetti A, Zanca A)., Feliciani, Claudio, Cozzani, Emanuele, Marzano, Angelo V., Caproni, Marzia, Di Zenzo, Giovanni, Calzavara-Pinton, Piergiacomo, La placa M, Patrizi a, Feliciani, C, Cozzani, E, Marzano, Av, Caproni, M, Di Zenzo, G, Calzavara-Pinton, P, 'Cutaneous Immunology' group of SIDeMaST (Alaibac, M, Antiga, E, Arisi, M, Assalve, D, Atzori, L, Babino, G, Balato, A, Balato, N, Barbagallo, T, Belloni Fortina, A, Berardesca, E, Berti, E, Borghi, A, Brazzelli, V, Brumana, Mb, Brusati, C, Caccavale, S, Caldarola, G, Campanati, A, Caposiena Caro, Rd, Coati, I, Conti, A, Costanzo, A, Dattola, A, De Simone, C, Di Cesare, A, Di Lernia, V, Di Zenzo, Gm, Fania, L, Filosa, G, Filotico, R, Fusano, M, Gerarda Galluccio, A, Ganzetti, G, Gisondi, P, Guarneri, F, Hansel, K, Javor, S, La Placa, M, Liberati, G, Lora, V, Merlo, G, Nasca, Mr, Nigro, A, Noal, C, Offidani, A, Papini, M, Parodi, A, Patrizi, A, Peserico, A, Piaserico, S, Prignano, F, Quaglino, P, Rossi, M, Satta, R, Schena, D, Sena, P, Simonetti, O, Stan, Tr, Stingeni, L, Talamonti, M, Tavecchio, S, Tognetti, L, Vascellaro, A, Vassallo, C, Venturini, M, Zampetti, A, and Zanca, A).

Subjects
Male, medicine.medical_specialty, Venereology, Pemphigus - Guidelines - Skin diseases, Vesiculobullous, Autoantibodies, Evidence-Based Medicine, Female, Humans, Italy, Pemphigus, Skin, Socio-culturale, Dermatology, Desmoglein, Pemphigu, 030207 dermatology & venereal diseases, 03 medical and health sciences, 0302 clinical medicine, Settore MED/35, Medicine, Skin pathology, skin and connective tissue diseases, Vesiculobullou, integumentary system, business.industry, Guideline, Evidence-based medicine, medicine.disease, Autoantibodie, 2708, 030220 oncology & carcinogenesis, Pemphigus - Guidelines - Skin disease, business, Blistering disease, Human
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 in collaboration with the European Academy of Dermatology and Venereology. It summarizes evidence-based and expert-based recommendations (S2 level).

Published
2018

2. Bullous pemphigoid: Italian guidelines adapted from the EDF/EADV guidelines

Author

Cozzani, Emanuele, Marzano, Angelo V, Caproni, Marzia, Feliciani, Claudio, Calzavara-Pinton, Piergiacomo, Alaibac, M, Antiga, E, Arisi, M, Assalve, D, Atzori, L, Babino, G, Balato, A, Balato, N, Barbagallo, T, Belloni Fortina, A, Berardesca, E, Berti, E, Borghi, A, Brazzelli, V, Brumana, Mb, Brusati, C, Caccavale, S, Caldarola, G, Calzavara-Pinton, P, Campanati, A, Caposiena Caro RD, Caproni, M, Coati, I, Conti, A, Costanzo, A, Cozzani, E, Dattola, A, De Simone, C, Di Cesare, A, Di Lernia, V, Di Zenzo GM, Fania, L, Filosa, G, Filotico, R, Fusano, M, Gerarda Galluccio, A, Ganzetti, G, Gisondi, P, Guarneri, F, Hansel, K, Javor, S, La Placa, M, Liberati, G, Lora, V, Marzano, Av, Merlo, G, Nasca, Mr, Nigro, A, Noal, C, Offidani, A, Papini, M, Parodi, A, Patrizi, A, Peserico, A, Piaserico, S, Prignano, F, Quaglino, P, Rossi, M, Satta, R, Schena, D, Sena, P, Simonetti, O, Stan, Tr, Stingeni, L, Talamonti, M, Tavecchio, S, Tognetti, L, Vascellaro, A, Vassallo, C, Venturini, M, Zampetti, A, Zanca, A., Cozzani, Emanuele, Marzano, Angelo V., Caproni, Marzia, Feliciani, Claudio, Calzavara-Pinton, Piergiacomo, Alaibac, Mauro, Antiga, Emiliano, Arisi, Mariachiara, Assalve, Danilo, Atzori, Laura, Babino, Graziella, Balato, Anna, Balato, Nicola, Barbagallo, Tania, Fortina, Anna Belloni, Berardesca, Enzo, Berti, Emilio, Borghi, Alessandro, Brazzelli, Valeria, Brumana, Marta B., Brusati, Carlo, Caccavale, Stefano, Caldarola, Giacomo, Campanati, Anna, Caro, Raffaele D. Caposiena, Coati, Ilaria, Conti, Andrea, Costanzo, Antonio, Dattola, Annunziata, De Simone, Clara, Di Cesare, Antonella, Di Lernia, Vito, Di Zenzo, Giovanni M., Fania, Luca, Filosa, Giorgio, Filotico, Raffaele, Fusano, Marta, Galluccio, Antonia Gerarda, Ganzetti, Giulia, Gisondi, Paolo, Guarneri, Fabrizio, Hansel, Katharina, Javor, Sanja, Laplaca, Michelangelo, Liberati, Giulia, Lora, Viviana, Merlo, Giulia, Nasca, Maria R., Nigro, Anna, Noal, Cecilia, Offidani, Annamaria, Papini, Manuela, Parodi, Aurora, Patrizi, Annalisa, Peserico, Andrea, Piaserico, Stefano, Prignano, Francesca, Quaglino, Pietro, Rossi, Mariateresa, Satta, Rosanna, Schena, Donatella, Sena, Paolo, Simonetti, Oriana, Stan, Teodora R., Stingeni, Luca, Talamonti, Marina, Tavecchio, Simona, Tognetti, Linda, Vascellaro, Annalisa, Vassallo, Camilla, Venturini, Marina, Zampetti, Anna, Zanca, Arianna, and La placa, Michelangelo

Subjects
Pemphigoid, Disease, Guideline, 030207 dermatology & venereal diseases, Pruritu, 0302 clinical medicine, Quality of life, Pemphigoid, Bullous, skin and connective tissue diseases, Evidence-Based Medicine, integumentary system, Bullous, Rare diseases, Bullous lesions, Infectious Diseases, Italy, 030220 oncology & carcinogenesis, Practice Guidelines as Topic, Bullou, Bullous pemphigoid, Human, medicine.medical_specialty, Venereology, Consensus, Socio-culturale, Consensu, Dermatology, Aged, Humans, Pruritus, Quality of Life, 2708, 03 medical and health sciences, Pemphigoid, Bullou, Settore MED/35, medicine, business.industry, Evidence-based medicine, medicine.disease, eye diseases, business, Rare disease
Abstract

Bullous pemphigoid is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or generalized bullous lesions. In up to 20% of affected patients blister may be completely absent, and only excoriations, prurigo-like lesions, eczematous lesions, urticated lesions, and/or infiltrated plaques are observed. The disease is significantly associated with neurological disorders. The morbidity of bullous pemphigoid and its impact on the quality of life are significant. So far, a limited number of national treatment guidelines have been proposed, but no common European consensus has emerged. This guideline for the treatment of bullous pemphigoid 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 summarizes evidence-based and expert-based recommendations (S2 level).

Published
2018

3. Interleukin-36 cytokines are overexpressed in the skin and sera of patients with bullous pemphigoid.

Author

Maglie R, Mercurio L, Morelli M, Madonna S, Salemme A, Baffa ME, Quintarelli L, Di Zenzo GM, Antiga E, and Albanesi C

Subjects
Humans, Cytokines metabolism, Skin metabolism, Interleukins metabolism, Inflammation metabolism, Autoantibodies, Autoantigens, Non-Fibrillar Collagens, Pemphigoid, Bullous, Psoriasis metabolism
Abstract

Bullous pemphigoid (BP) is an autoimmune bullous disease, characterized by autoantibodies targeting BP180 and BP230. The role of interleukin (IL)-36, a potent chemoattractant for granulocytes, in BP remains elusive.The expression of IL-36 cytokines (IL-36α, β, γ) and their antagonists (IL-36Ra and IL-38) was analysed in the skin and serum samples of patients with BP (n = 31), psoriasis (n = 10) and healthy controls (HC) (n = 14) by quantitative polymerase chain reaction and enzyme linked immunosorbent assay, respectively. Skin and serum levels of all cytokines were correlated with the Bullous Pemphigoid Disease Area Index (BPDAI) score and with the serum concentration of pathogenic antibodies.IL-36α, IL-36β, IL-36γ and IL-36Ra were significantly (p < 0.05) overexpressed in BP skin compared to HC, without remarkable differences relative to psoriasis skin. The expression of IL-38 was significantly (p < 0.05) higher in BP compared to psoriasis skin.IL-36α and γ, but not β, serum concentrations were significantly (p < 0.05) higher in BP compared to HC. IL-36γ was significantly (p < 0.05) more expressed in the serum of psoriasis patients than BP. The serum concentration of IL-36Ra and IL-38 were similar between BP and HC, while IL-38 serum levels were significantly (p < 0.05) higher in BP compared to psoriasis patients. Serum IL-36α correlated significantly with BPDAI (r = 0.5 p = 0.001).IL-36 agonists are increased in BP patients, both locally and systemically. Serum IL-36α might represent a potential biomarker for BP. An inefficient balance between IL-36 agonists and antagonists is likely to occur during BP inflammation., (© 2023 The Authors. Experimental Dermatology published by John Wiley & Sons Ltd.)

Published
2023
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4. Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV).

Author

Borradori L, Van Beek N, Feliciani C, Tedbirt B, Antiga E, Bergman R, Böckle BC, Caproni M, Caux F, Chandran NS, Cianchini G, Daneshpazhooh M, De D, Didona D, Di Zenzo GM, Dmochowski M, Drenovska K, Ehrchen J, Goebeler M, Groves R, Günther C, Horvath B, Hertl M, Hofmann S, Ioannides D, Itzlinger-Monshi B, Jedličková J, Kowalewski C, Kridin K, Lim YL, Marinovic B, Marzano AV, Mascaro JM, Meijer JM, Murrell D, Patsatsi K, Pincelli C, Prost C, Rappersberger K, Sárdy M, Setterfield J, Shahid M, Sprecher E, Tasanen K, Uzun S, Vassileva S, Vestergaard K, Vorobyev A, Vujic I, Wang G, Wozniak K, Yayli S, Zambruno G, Zillikens D, Schmidt E, and Joly P

Subjects
Adrenal Cortex Hormones therapeutic use, Aged, Blister drug therapy, Humans, Quality of Life, Dermatology, Pemphigoid, Bullous diagnosis, Pemphigoid, Bullous drug therapy, Venereology
Abstract

Background: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo-like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life., Objectives and Methodology: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology sought to update the guidelines for the management of BP based on new clinical information, and new evidence on diagnostic tools and interventions. The recommendations are either evidence-based or rely on expert opinion. The degree of consent among all task force members was included., Results: Treatment depends on the severity of BP and patients' comorbidities. High-potency topical corticosteroids are recommended as the mainstay of treatment whenever possible. Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of contraindications or resistance to corticosteroids, immunosuppressive therapies, such as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be recommended. The use of doxycycline and dapsone is controversial. They may be recommended, in particular, in patients with contraindications to oral corticosteroids. B-cell-depleting therapy and intravenous immunoglobulins may be considered in treatment-resistant cases. Omalizumab and dupilumab have recently shown promising results. The final version of the guideline was consented to by several patient organizations., Conclusions: The guidelines for the management of BP were updated. They summarize evidence- and expert-based recommendations useful in clinical practice., (© 2022 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)

Published
2022
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