Incidence and Mortality of Pemphigus in France

Jelti, L.; Cordel, N.; Gillibert, André; Lacour, Jean‐Philippe; Uthurriague, C.; Doutre, M.-S.; Delaporte, E.; Duvert‐Lehembre, S.; Quéreux, G.; Dupuy, Alain; Adamski, H.; Bédane, C.; Miséry, Laurent; Thomas, C. Abasq; Fleuret, C.; Bernard, Philippe; Chaby, G.; D’Incan, M.; Verneuil, Laurence; Litrowski, N.; Joly, P.

doi:10.1016/j.jid.2018.07.042

Cited by 39 publications

(37 citation statements)

References 10 publications

(11 reference statements)

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“…5,6 Pemphigus is rare, and its incidence has been estimated to about 2 new patients per 1 million inhabitants per year in Central Europe. 7,8 Two main clinical variants are known, pemphigus vulgaris (PV) and pemphigus foliaceus (PF). The pathogenic role of anti-desmoglein 1 and 3 IgG has been clearly established since the injection of patients' sera or affinity-purified IgG from pemphigus sera into neonatal mice reproduces clinically and immunopathologically the cardinal symptoms of pemphigus within 24 h. 1,5 .…”

Section: Introductionmentioning

confidence: 99%

“…Pathophysiologically, the underlying intraepithelial blister formation is caused by IgG autoantibodies against the desmosomal adhesion proteins, desmoglein 3 (Dsg 3) and/or desmoglein 1 (Dsg1), on the cell surface of epidermal keratinocytes 5,6 . Pemphigus is rare, and its incidence has been estimated to about 2 new patients per 1 million inhabitants per year in Central Europe 7,8 . Two main clinical variants are known, pemphigus vulgaris (PV) and pemphigus foliaceus (PF).…”

Section: Introductionmentioning

confidence: 99%

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Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV)

Joly

Hórvath

Patsatsi

et al. 2020

Acad Dermatol Venereol

209

302

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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, pemphigus was almost always fatal. Due to its rarity, only few randomized controlled therapeutic trials are available. Recently, rituximab has been approved as first‐line treatment for moderate and severe pemphigus vulgaris in Europe and the United States. Objectives The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology (EADV) has initiated a throughout update of the guideline for the management of patients with pemphigus. Results The guidelines for the management of pemphigus were updated, and the degree of consent among all task force members was included. The final version of the guideline was consented by the European Dermatology Forum (EDF) and several patient organizations.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV)

Joly

Hórvath

Patsatsi

et al. 2020

Acad Dermatol Venereol

209

302

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“…The epidemiology of paraneoplastic pemphigus (PNP), a rare pemphigus variant accounting for about 5% of pemphigus cases ( Jelti et al., 2019 ), is summarized later. IgA pemphigus, which was reviewed in Hashimoto et al.…”

Section: Introductionmentioning

confidence: 99%

Epidemiology of Pemphigus

Kridin

Schmidt

2021

JID Innovations

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Pemphigus is an epidemiologically heterogeneous group of autoimmune bullous diseases comprising pemphigus vulgaris (PV), pemphigus foliaceus, paraneoplastic pemphigus, IgA pemphigus, and pemphigus herpetiformis. Recently, our knowledge about the frequency of pemphigus, which is highly variable between different populations, has considerably expanded, and the first non-HLA genes associated with PV have been identified. In addition, a variety of comorbidities, including other autoimmune diseases, hematological malignancies, and psoriasis, have been described in this variant. Here, initial data about the impact of COVID-19 on this fragile patient population are discussed and perspectives for future epidemiological studies are outlined.

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“…2 The incidence of pemphigus varies between 0.5-34 cases/million/year and there is significant geographic variation in incidence rates. 3 Pemphigus vulgaris (PV) is the most common variant in most countries and is characterised by anti-Dsg-3 and/or Dsg-1 antibodies. Dsg-1 is expressed predominantly in the uppermost layers of the epidermis while Dsg-3 is expressed predominantly in mucosal tissues but also in the lower epidermis.…”

Section: Pemphigusmentioning

confidence: 99%

Immunobullous disease

Tull

Benton

2021

Clinical Medicine

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Immunobullous diseases are blistering cutaneous disorders that are caused by pathogenic antibodies binding to protein targets within the skin. There are a range of immunobullous disorders with characteristic morphology that relates to the structural properties of the target protein. In this article we will describe the pathogenesis, clinical features and treatment of the most common immunobullous disorders.

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Incidence and Mortality of Pemphigus in France

Cited by 39 publications

References 10 publications

Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV)

Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV)

Epidemiology of Pemphigus

Immunobullous disease

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