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Pemphigus. S2 Guideline for diagnosis and treatment – guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV)

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TLDR
Pemphigus encompasses a group of life‐threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin that was almost fatal before the era of immunosuppressive treatment.
Abstract
BACKGROUND Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, the prognosis of pemphigus was almost fatal. Due to its rarity, only few prospective controlled therapeutic trials are available. OBJECTIVES For this reason, a group of European dermatologists with a long-standing interest and expertise in basic and clinical pemphigus research has sought to define diagnostic and therapeutic guidelines for the management of patients with pemphigus. RESULTS This group identified the statements of major agreement or disagreement regarding the diagnostic and therapeutic management of pemphigus. The revised final version of the pemphigus guideline was finally passed on to the European Dermatology Forum (EDF) for a final consensus with the European Academy of Dermatology and Venereology (EADV) and the European Union of Medical Specialists (UEMS).

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Citations
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Diagnosis and management of pemphigus: Recommendations of an international panel of experts

Dedee F. Murrell, +60 more
TL;DR: Recommendations from a subsequent Delphi consensus to broaden the generalizability of recommendations includes intravenous CD20 inhibitors as a first line therapy option for moderate to severe pemphigus.
References
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Journal ArticleDOI

Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion

TL;DR: It is demonstrated that a novel epithelial cadherin is the target of autoantibodies in PV, and the deduced amino acid sequence of PVA was unique but showed significant homology with members of the cadher in family of Ca(2+)-dependent cell adhesion molecules.
Journal ArticleDOI

Recommendations for the Prevention and Treatment of Glucocorticoid-Induced Osteoporosis

TL;DR: Treatment with a bisphosphonate is recommended to prevent bone loss in all men and postmenopausal women in whom long-term glucocorticoid treatment at > or =5 mg/day is being initiated, as well as in men andPost menopausal women receiving long- term glucocORTicoids in whom the BMD T-score at either the lumbar spine or the hip is below normal.
Journal ArticleDOI

Induction of Pemphigus in Neonatal Mice by Passive Transfer of IgG from Patients with the Disease

TL;DR: This study strongly supports the proposed role of pemphigus autoantibodies in the pathogenesis of p emphigu vulgaris in human beings and demonstrates that pemPHigus can be passively transferred to laboratory animals.
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Diagnosis and management of pemphigus: Recommendations of an international panel of experts

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