Persistence of antienvoplakin and antiperiplakin antibodies in a patient with paraneoplastic pemphigus 20 years after remission

Riera‐Monroig, Josep; Iranzo, Pilar; Ishii, Norito; Hashimoto, Takashi; Mascaró, José M.

doi:10.1111/bjd.18534

Cited by 3 publications

(5 citation statements)

References 8 publications

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“…44,[124][125][126][127] Early detection and radical resection of tumours such as Castleman's disease or thymoma have occasionally been shown to have a beneficial effect and lead to PNP/PAMS resolution 48,50 with long-term survival. 128 There is no evidence supporting the use of any specific therapy due to the rarity of the condition. 129 However, systemic corticosteroids (prednisolone) 0.5 mg/kg to 1.5 mg/kg/ day in combination with steroid-sparing agents have been widely used.…”

Section: Th Er a Pymentioning

confidence: 99%

“…The treatment of the underlying malignancy is always recommended, as management of the underlying neoplasm can result in PNP/PAMS improvement 44,124–127 . Early detection and radical resection of tumours such as Castleman's disease or thymoma have occasionally been shown to have a beneficial effect and lead to PNP/PAMS resolution 48,50 with long‐term survival 128 …”

Section: Therapymentioning

confidence: 99%

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S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)

Antiga

Bech

Maglie

et al. 2023

Acad Dermatol Venereol

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Background: Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans.Objectives: These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. Results: Chronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. Conclusions: These are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies.

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Section: Th Er a Pymentioning

confidence: 99%

Section: Therapymentioning

confidence: 99%

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)

Antiga

Bech

Maglie

et al. 2023

Acad Dermatol Venereol

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“…6 The eradication of the underlying tumour may have some benefit in the mucocutaneous lesions; nonetheless, even after complete resection, the autoimmune process may remain active for many years. 16 Moreover, there are reports where the pulmonary involvement not only persisted but progressed after tumour resection. 13,14 Systemic therapy with thalidomide, lenalidomide, bortezomib, cytotoxic chemotherapy, rituximab or siltuximab (anti-interleukin-6 monoclonal antibody) may be considered for treatment of residual disease.…”

Section: Discussionmentioning

confidence: 99%

“…Whether prognosis differs between unicentric and multicentric disease is uncertain 6 . The eradication of the underlying tumour may have some benefit in the mucocutaneous lesions; nonetheless, even after complete resection, the autoimmune process may remain active for many years 16 . Moreover, there are reports where the pulmonary involvement not only persisted but progressed after tumour resection 13,14 .…”

Section: Discussionmentioning

confidence: 99%

Paraneoplastic pemphigus associated with Castleman's disease

Relvas

Xará

Lucas

et al. 2023

J Paediatrics Child Health

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“…Thus, complete resection of her UCD may have limited the severity of the BO. Long‐term survival in PNP is rare, and autoantibodies can persist for many years after treatment of the underlying neoplasm 27 . In prior cases, patients with prolonged survival either lacked pulmonary involvement or underwent lung transplantation following the development of BO.…”

Section: Discussionmentioning

confidence: 99%

Paraneoplastic pemphigus associated with Castleman disease: A multicenter case series

Barry

Plumptre

Bazewicz

et al. 2022

Pediatric Dermatology

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Introduction: Paraneoplastic pemphigus (PNP) is a rare, often fatal, autoimmune blistering disease of the skin and mucous membranes. In children, PNP is frequently associated with Castleman disease (CD). This series describes five cases of PNP associated with CD.Methods: Data were collected retrospectively from the medical records of patients with a diagnosis of PNP and CD from January 2013 to June 2022. Patients ≤22 years old with clinical and immunopathologic evidence of PNP were included; CD was diagnosed histopathologically.Results: Two children, two adolescents, and one young adult (two males, three females) were included. The average age at disease presentation was 11.8 years (range: 7-22 years). Oral (n = 5) and anogenital (n = 3) mucositis were common. Four patients had "unicentric" CD (UCD); one patient had "multicentric" CD (MCD). Castleman tumors were in the retroperitoneum (n = 4) or axilla (n = 1). One patient had myasthenia gravis without thymoma. Three patients had bronchiolitis obliterans (BO).Three patients had complete resection of their CD; two had partial resection. Three patients remain alive with a median follow-up of 13 months (range: 12 months to 13 years); two are clinically stable with resolution of mucocutaneous lesions; one has persistent BO requiring ongoing ventilatory support. Patients who remain alive had UCD with complete resection; all deceased patients had partial resection and BO. Conclusion:Most patients had UCD, and the retroperitoneum was the most common location. Patients with MCD, incomplete resection, and BO died; patients with UCD and complete resection remain alive, even in the setting of BO. Consideration of PNP is critical when pediatric patients present with mucositis as PNP may be clinically indistinguishable from more common causes of mucositis.

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Persistence of antienvoplakin and antiperiplakin antibodies in a patient with paraneoplastic pemphigus 20 years after remission

Cited by 3 publications

References 8 publications

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)

Paraneoplastic pemphigus associated with Castleman's disease

Paraneoplastic pemphigus associated with Castleman disease: A multicenter case series

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