Pemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment

Autoimmune bullous disorders are a heterogeneous spectrum of skin disorders characterized by the production of autoantibodies against adhesion molecules of the skin. The 2 major groups of diseases are “pemphigus diseases” and “autoimmune bullous diseases of the pemphigoid type.” Pemphigus diseases...

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Autores principales:	Vito Di Lernia, Dahiana M. Casanova, Mohamad Goldust, Cinzia Ricci
Formato:	article
Lenguaje:	EN
Publicado:	Mattioli1885 2020
Materias:	pemphigus pemphigoid autoimmune bullous disorder Dermatology RL1-803
Acceso en línea:	https://doaj.org/article/a884caebaaca46a9b8f8201ed18baa71
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spelling	oai:doaj.org-article:a884caebaaca46a9b8f8201ed18baa712021-11-17T08:28:44ZPemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment 10.5826/dpc.1003a502160-9381https://doaj.org/article/a884caebaaca46a9b8f8201ed18baa712020-06-01T00:00:00Zhttp://dpcj.org/index.php/dpc/article/view/1099https://doaj.org/toc/2160-9381 Autoimmune bullous disorders are a heterogeneous spectrum of skin disorders characterized by the production of autoantibodies against adhesion molecules of the skin. The 2 major groups of diseases are “pemphigus diseases” and “autoimmune bullous diseases of the pemphigoid type.” Pemphigus diseases are a group of autoimmune blistering diseases of the skin and mucous membranes characterized by intraepithelial cleft and acantholysis. The main subtypes of pemphigus include pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. Diagnosis is based on clinical manifestations and confirmed with histological, immunofluorescence, and serological testing. Recently multivariant enzyme‐linked immunosorbent assay systems have been developed as practical screening tools for patients with suspected autoimmune bullous dermatoses. The current first-line treatment of pemphigus is based on systemic corticosteroids that are often combined with immunosuppressive adjuvants, such as azathioprine, mycophenolate mofetil, and the anti-CD20 monoclonal antibody rituximab, usually at initiation of treatment. Rituximab efficacy is higher when it is administered early in the course of the disease. Therefore, it should be used as first-line treatment to improve efficacy and reduce cumulative doses of corticosteroids and their side effects. Treatment of bullous pemphigoid is based on disease extension. Localized and mild forms can be treated with superpotent topical corticosteroids or with nonimmunosuppressive agents. In patients with generalized disease or whose disease is resistant to the treatments described above, systemic corticosteroids are preferred and effective. Adjuvant immunosuppressants are often combined with steroids for their steroid-sparing effect. Vito Di LerniaDahiana M. CasanovaMohamad GoldustCinzia RicciMattioli1885articlepemphiguspemphigoidautoimmunebullousdisorderDermatologyRL1-803ENDermatology Practical & Conceptual, Vol 10, Iss 3 (2020)
institution	DOAJ
collection	DOAJ
language	EN
topic	pemphigus pemphigoid autoimmune bullous disorder Dermatology RL1-803
spellingShingle	pemphigus pemphigoid autoimmune bullous disorder Dermatology RL1-803 Vito Di Lernia Dahiana M. Casanova Mohamad Goldust Cinzia Ricci Pemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment
description	Autoimmune bullous disorders are a heterogeneous spectrum of skin disorders characterized by the production of autoantibodies against adhesion molecules of the skin. The 2 major groups of diseases are “pemphigus diseases” and “autoimmune bullous diseases of the pemphigoid type.” Pemphigus diseases are a group of autoimmune blistering diseases of the skin and mucous membranes characterized by intraepithelial cleft and acantholysis. The main subtypes of pemphigus include pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. Diagnosis is based on clinical manifestations and confirmed with histological, immunofluorescence, and serological testing. Recently multivariant enzyme‐linked immunosorbent assay systems have been developed as practical screening tools for patients with suspected autoimmune bullous dermatoses. The current first-line treatment of pemphigus is based on systemic corticosteroids that are often combined with immunosuppressive adjuvants, such as azathioprine, mycophenolate mofetil, and the anti-CD20 monoclonal antibody rituximab, usually at initiation of treatment. Rituximab efficacy is higher when it is administered early in the course of the disease. Therefore, it should be used as first-line treatment to improve efficacy and reduce cumulative doses of corticosteroids and their side effects. Treatment of bullous pemphigoid is based on disease extension. Localized and mild forms can be treated with superpotent topical corticosteroids or with nonimmunosuppressive agents. In patients with generalized disease or whose disease is resistant to the treatments described above, systemic corticosteroids are preferred and effective. Adjuvant immunosuppressants are often combined with steroids for their steroid-sparing effect.
format	article
author	Vito Di Lernia Dahiana M. Casanova Mohamad Goldust Cinzia Ricci
author_facet	Vito Di Lernia Dahiana M. Casanova Mohamad Goldust Cinzia Ricci
author_sort	Vito Di Lernia
title	Pemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment
title_short	Pemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment
title_full	Pemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment
title_fullStr	Pemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment
title_full_unstemmed	Pemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment
title_sort	pemphigus vulgaris and bullous pemphigoid: update on diagnosis and treatment
publisher	Mattioli1885
publishDate	2020
url	https://doaj.org/article/a884caebaaca46a9b8f8201ed18baa71
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Pemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment

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