Cutaneous type of pemphigus vulgaris

Gheisari, Mehdi; Shahidi‐Dadras, Mohammad; Nasiri, Soheila; Dargah, Samaneh Mohseni; Dadkhahfar, Sahar; Abdollahimajd, Fahimeh

doi:10.1016/j.jaad.2019.12.065

Cited by 7 publications

(8 citation statements)

References 5 publications

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“…Despite the serological positivity for both anti-Dsg1 and anti-Dsg3 IgG, the patient never showed mucosal involvement, while the cutaneous lesions were clinically more compatible with PV than with pemphigus foliaceous. Interestingly, other authors hypothesized the existence of a “cutaneous-type” PV, describing similar cases where the absence of mucosal lesions could be due to the low pathogenetic activity of anti-Dsg3 not sufficient to induce blistering as in the mucocutaneous or mucosal-dominant phenotypes of PV ( 10 , 11 ).…”

Section: Discussionmentioning

confidence: 99%

Case Report: Management of Malignancy-Exacerbated Pemphigus Vulgaris During COVID-19 Pandemic

et al. 2021

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Pemphigus vulgaris is an intraepidermal autoimmune mucocutaneous blistering disease whose etiopathogenesis includes various trigger factors, i.e., drugs and malignancies. We present a case of malignancy-exacerbated pemphigus vulgaris which required a careful diagnostic process in order to rule out paraneoplastic pemphigus, along with the challenges posed by the need of treating both cutaneous and oncologic diseases. Possible post-operative complications post-poned the start of first-line immunosuppressive treatment of pemphigus. Moreover, the infective risks had to be minimized during the peak of the COVID-19 pandemic in Italy. Intravenous immunoglobulins were chosen as “bridge” therapy before the tumor surgical excision, followed by rituximab in post-operative phase.

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Section: Discussionmentioning

confidence: 99%

Case Report: Management of Malignancy-Exacerbated Pemphigus Vulgaris During COVID-19 Pandemic

et al. 2021

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“…Dsg3 antibodies alone do not lead to cutaneous lesions thanks to dsg1 expression which preserves epidermal stability (compensation theory) 40 . In some cases, patients with both dsg3‐and dsg1‐antibodies present with skin‐limited PV (cutaneous‐PV) 41 . Antibodies to other desmosomal proteins, particularly dsc3, may be found less frequently 42 …”

Section: Pemphigus Vulgarismentioning

confidence: 99%

Pemphigus and pemphigoids: Clinical presentation, diagnosis and therapy

Didona,

Schmidt,

Maglie

et al. 2023

J Deutsche Derma Gesell

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SummaryPemphigus and pemphigoid are two potentially life‐threatening groups of autoimmune diseases, characterized by autoantibodies targeting structural components of desmosomes or hemidesmosomes, respectively. Affected patients typically show itchy/painful plaques or blistering skin lesions and/or impairing mucosal blistering and erosions, which may strongly impact their quality of life. Since the milestone work of Walter Lever in 1953, who differentiated these two groups of diseases by histopathological analysis of the level of antibody‐mediated skin cleavage, enormous progresses occurred. Achievements made in laboratory diagnostics now allow to identify antigen specific structural proteins of the skin that are targeted by pathogenic autoantibodies. These progresses were accompanied by an increased understanding of the pathogenesis of these diseases thanks to the establishment of animal models reproducing disease and on studies on skin and blood of affected individuals, which have been leading to novel and disease‐specific treatments. Yet, given their phenotypical overlap with more common dermatological diseases, correct diagnosis and appropriate treatment are often delayed, in some cases leading to irreversible sequelae, including organ dysfunction (i.e., loss of vision in mucous membrane pemphigoid). Here, we provide a concise overview of the clinical appearance, diagnosis and therapeutic management of pemphigus and pemphigoid diseases.

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“…Anti‐Dsg3‐Antikörper allein führen nicht zu Hautläsionen, da durch die Dsg1‐Expression die epidermale Stabilität erhalten bleibt (Kompensationstheorie) 40 . In einigen Fällen führt auch das gleichzeitige Vorliegen von Anti‐Dsg3‐ und ‐Dsg1‐Antikörper zu einer kutanen Manifestation, während die Schleimhäute ausgespart bleiben 41 . In seltenen Fällen können auch AK gegenüber andere desmosomale Proteinen, vor allem Anti‐Dsc‐3‐Antikörper, nachgewiesen werden 42 …”

Section: Pemphigus Vulgarisunclassified

Pemphigus‐ und Pemphigoid‐Erkrankungen: Klinik, Diagnostik und Therapie

Didona,

Schmidt,

Maglie

et al. 2023

J Deutsche Derma Gesell

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ZusammenfassungPemphigus und Pemphigoid sind seltene Autoimmunkrankheiten der Haut mit potenziell lebensbedrohlichem Verlauf. Autoantikörper gegen epidermale und junktionale Strukturproteine (Desmosomen sowie Hemidesmosomen) führen bei Betroffenen typischerweise zu juckenden, schmerzhaften Plaques oder Blasen an der Haut und/oder Blasenbildung und Erosionen der Schleimhäute mit möglicher Einschränkung der Lebensqualität. Seit der bahnbrechenden Arbeit von Walter Lever im Jahr 1953, dem es gelang, mittels histopathologischer Untersuchung diese beiden Krankheitsgruppen anhand des Musters der Antikörper‐vermittelten Blasenbildung zu differenzieren, wurden enorme Fortschritte im Verständnis der Erkrankungen erzielt. Die Errungenschaften in der Labordiagnostik ermöglichten die Identifikation von Zielstrukturen zur präzisen Unterscheidung verschiedener Varianten der bullösen Autoimmunerkrankungen. Diese Fortschritte gingen dank der Entwicklung von Tiermodellen mit einem besseren Verständnis der Pathogenese einher. Außerdem haben Studien an Haut und Blut betroffener Patienten zu neuen und krankheitsspezifischen Behandlungen geführt. Aufgrund ihrer Seltenheit und der klinischen Ähnlichkeit mit anderen dermatologischen Erkrankungen verzögern sich die korrekte Diagnosestellung und die Einleitung einer entsprechenden Therapie häufig, was in einigen Fällen zu irreversiblen Folgeerscheinungen, einschließlich Funktionsstörungen von Organen (zum Beispiel Verlust des Sehvermögens beim Schleimhautpemphigoid) führt. Wir geben hier einen Überblick über das klinische Erscheinungsbild, den Diagnosealgorithmus und das therapeutische Management von Pemphigus‐ und Pemphigoid‐Erkrankungen.

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Cutaneous type of pemphigus vulgaris

Abstract: Extended wear bandage contact lenses decrease pain and preserve vision in patients with epidermolysis bullosa: case series and review of literature.

Cited by 7 publications

References 5 publications

Case Report: Management of Malignancy-Exacerbated Pemphigus Vulgaris During COVID-19 Pandemic

Case Report: Management of Malignancy-Exacerbated Pemphigus Vulgaris During COVID-19 Pandemic

Pemphigus and pemphigoids: Clinical presentation, diagnosis and therapy

Pemphigus‐ und Pemphigoid‐Erkrankungen: Klinik, Diagnostik und Therapie

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