Paraneoplastic pemphigus associated with Castleman's disease and thyroid carcinoma with anti‐BP180, BP230 and antidesmocollin autoantibodies

Velasco‐Tamariz, Virginia; Calleja‐Algarra, Alba; Pérez, Sara; Atauri, Álvaro Gimeno Díaz de; Gómez, Cristina de Manuel; Caldevilla-Asenjo, Laura; Ishii, Norito; Hashimoto, Takashi; Peralto, J.L. Rodríguez; Romero, Pablo Luis Ortiz

doi:10.1111/ddg.14393

Cited by 6 publications

(11 citation statements)

References 7 publications

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“…Paraneoplastic pemphigus is inevitably associated with neoplasia, usually thymoma or hematologic malignancy, and shows an exceptionally pronounced involvement of the oral mucosa, usually combined with involvement of the lips. 13 In pemphigus foliaceus, only the skin is affected showing erosions and plaques reminiscent of flaky pastry predominantly in seborrheic areas of trunk and head. IgA pemphigus is a very rare entity and represents a differential diagnosis of pustular psoriasis and subcorneal pustular dermatosis -clinical features are pustules appearing on the body.…”

Section: Pemphigus Diseasesmentioning

confidence: 99%

“…This condition is referred to as mucocutaneous PV (Figure 1b). Paraneoplastic pemphigus is inevitably associated with neoplasia, usually thymoma or hematologic malignancy, and shows an exceptionally pronounced involvement of the oral mucosa, usually combined with involvement of the lips 13 . In pemphigus foliaceus, only the skin is affected showing erosions and plaques reminiscent of flaky pastry predominantly in seborrheic areas of trunk and head.…”

Section: Clinical Featuresmentioning

confidence: 99%

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Autoimmune bullous dermatoses

Holtsche

Boch

Schmidt

2023

J Deutsche Derma Gesell

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SummaryAutoimmune bullous dermatoses (AIBD) are a heterogeneous group of about a dozen diseases characterized clinically by erosions and blisters and immunopathologically by autoantibodies against structural proteins of the skin or transglutaminase 2/3. The diagnosis of AIBD has made tremendous progress in the last decade due to the availability of standardized serological assays that, knowing the clinical picture, allow the diagnosis in the vast majority of patients. The development of various in vitro and in vivo models of the most common AIBD, namely, bullous pemphigoid, pemphigus vulgaris, mucous membrane pemphigoid, and the rare epidermolysis bullosa acquisita, allows identification of key molecules and inflammatory pathways as well as preclinical evaluation of the effect of new anti‐inflammatory agents. The approval of rituximab for moderate and severe pemphigus vulgaris and the development of national and international guidelines for the most common AIBD have considerably advanced the care of these patients. Nevertheless, the limited therapeutic armamentarium is the main challenge for the management of AIBD. Several phase II and III randomized controlled clinical trials provide hope for new, effective, and safe therapeutic options in the coming years. This review summarizes the epidemiology, clinic, diagnosis, pathophysiology, and therapy of AIBD and gives an outlook on both current diagnostic and therapeutic needs as well as future developments.

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Section: Pemphigus Diseasesmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

Autoimmune bullous dermatoses

Holtsche

Boch

Schmidt

2023

J Deutsche Derma Gesell

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“…Einen ausgesprochen starker Befall der Mundschleimhaut meist kombiniert mit Lippenbeteiligung zeigt der paraneoplastische Pemphigus, der obligat mit einer Neoplasie, meist einem Thymom oder einem hämatologischen Malignom, assoziiert ist. 13 Beim ausschließlich die Haut betreffenden Pemphigus foliaceus imponieren Erosionen und blätterteigartige Plaques hauptsächlich in den seborrhoischen Arealen an Stamm und Kopf. Der sehr seltene IgA-Pemphigus stellt eine Differenzialdiagnose der pustulösen Psoriasis und subkornealen Pustulose dar -klinisch treten Pusteln am Körper auf.…”

Section: Pemphigus-erkrankungenunclassified

“…Bei etwa 50% der PV‐Patienten kommt es zusätzlich zu schlaffen Blasen und Erosionen an der Haut, man spricht dann von einem mukokutanen PV (Abbildung 1b). Einen ausgesprochen starker Befall der Mundschleimhaut meist kombiniert mit Lippenbeteiligung zeigt der paraneoplastische Pemphigus, der obligat mit einer Neoplasie, meist einem Thymom oder einem hämatologischen Malignom, assoziiert ist 13 . Beim ausschließlich die Haut betreffenden Pemphigus foliaceus imponieren Erosionen und blätterteigartige Plaques hauptsächlich in den seborrhoischen Arealen an Stamm und Kopf.…”

Section: Klinikunclassified

Bullöse Autoimmundermatosen

Holtsche

Boch

Schmidt

2023

J Deutsche Derma Gesell

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ZusammenfassungBullöse Autoimmundermatosen (BAID) sind eine heterogene Gruppe von etwa einem Dutzend Erkrankungen, die klinisch durch Erosionen und Blasen und immunpathologisch durch Autoantikörper gegen Strukturproteine der Haut oder Transglutaminase 2/3 charakterisiert sind. Die Diagnostik der BAID hat in der letzten Dekade enorme Fortschritte gemacht durch die Verfügbarkeit standardisierter serologischer Nachweismethoden, welche in Kenntnis des klinischen Bildes die Diagnose bei der überwiegenden Mehrheit der Patienten erlaubt. Durch die Entwicklung verschiedener In‐vitro‐ und In‐vivo‐Modelle zu den häufigsten BAID, zum Beispiel bullöses Pemphigoid, Pemphigus vulgaris, Schleimhautpemphigoid sowie der seltenen Epidermolysis bullosa acquisita, ist es möglich, Schlüsselmoleküle und Entzündungswege zu identifizieren und die Wirkung neuer antientzündlicher Therapien präklinisch zu evaluieren. Die Zulassung von Rituximab für den moderaten und schweren Pemphigus vulgaris und die Entwicklung nationaler und internationaler Leitlinien für die häufigsten BAID stellt einen beträchtlichen Fortschritt in der Versorgung dieser Patienten dar. Trotzdem ist das limitierte therapeutische Armamentarium die größte Herausforderung für das Management der BAID. Mehrere randomisierte kontrollierte klinische Studien der Phasen II und III geben jedoch Anlass zur Hoffnung, dass in den nächsten Jahren neue wirksame und sichere Therapieoptionen zur Verfügung stehen werden. Die vorliegende Übersichtsarbeit fasst Epidemiologie, Klinik, Diagnostik, Pathophysiologie und Therapie der BAID zusammen und gibt einen Ausblick auf den aktuellen diagnostischen und therapeutischen Bedarf sowie zukünftige Entwicklungen.

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“…In children, PNP is most frequently seen in the setting of Castleman's disease, a rare, benign lymphoproliferative disorder, though it is almost always associated with myeloproliferative and lymphoproliferative disorders in adults [45,46,47 & ,48]. PNP presents as painful stomatitis with oral and vermillion lip ulcers followed by variable cutaneous eruptions which can mimic herpes simplex, Steven-Johnson syndrome, lupus, or another pemphigus [45,46,48]. One 2020 review [45] suggested children may be more likely to have lichenoid cutaneous eruptions over bullous eruptions due to higher tendencies toward cell-mediated cytotoxic disease rather than humoral-mediated disease, which is common in adults [49 ].…”

Section: Paraneoplastic Syndromesmentioning

confidence: 99%

Pearls and updates: cutaneous signs of systemic malignancy

Paquette

Cotter

Huang

2022

Current Opinion in Pediatrics

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Purpose of reviewChildren with systemic malignancies can present with concomitant dermatological manifestations. Cutaneous findings can occur before, during or after diagnosis. Recognizing these features can aid in diagnosis, inform prognosis, and help determine appropriate treatment. Following a review of the literature published over the past two years, we provide an update on cutaneous signs of pediatric systemic malignancies, concentrating on; leukemia cutis, lymphoma cutis, neuroblastoma, sarcomas, Langerhans cell histiocytosis and paraneoplastic syndromes. Recent findingsAuthors highlight the persistently heterogeneous features of cutaneous manifestations of systemic malignancy. Findings are often nonspecific, and a definitive diagnosis requires skin biopsy with immunophenotyping. Several studies describe dermoscopy features, demonstrating this as a useful tool in clinical evaluation. Genetic mutations underlying the pathogenesis of disease continue to be elucidated. Further, advances in medical treatment led to improved prognosis in many systemic malignancies, with early and aggressive treatment heralding better outcomes. SummaryComprehensive cutaneous evaluation alongside thorough clinical history and review of systems remains of paramount importance as dermatological manifestations of systemic malignancy are notoriously variable with a shared feature of often appearing benign but persisting despite usual treatment. Urgent referral to dermatology is recommended when suspicion for any cutaneous presentation of malignancy arises.

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Paraneoplastic pemphigus associated with Castleman's disease and thyroid carcinoma with anti‐BP180, BP230 and antidesmocollin autoantibodies

Cited by 6 publications

References 7 publications

Autoimmune bullous dermatoses

Autoimmune bullous dermatoses

Bullöse Autoimmundermatosen

Pearls and updates: cutaneous signs of systemic malignancy

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