A rare association of paraneoplastic pemphigus with gastric signet cell ring carcinoma

Lepekhova, Anfisa A.; Olisova, Olga Yu.; Teplyuk, N. P.; Zolotenkov, D.D.; Allenova, Anastasiia

doi:10.1111/ajd.12963

Cited by 1 publication

(2 citation statements)

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“…23,24 They can have epithelial or mesenchymal origin in about 9% and 6% of the cases, respectively. 24,[31][32][33][34][35] PNP/PAMS may rarely be triggered or exacerbated by either certain chemotherapy drugs (e.g. fludarabin, bendamustine and cyclophosphamide) [36][37][38][39] or by radiotherapy.…”

Section: Introductionmentioning

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: Introductionmentioning

confidence: 99%

“…Solid tumours have been found in 14.8%–17% of PNP/PAMS patients 23,24 . They can have epithelial or mesenchymal origin in about 9% and 6% of the cases, respectively 24,31–35 . PNP/PAMS may rarely be triggered or exacerbated by either certain chemotherapy drugs (e.g.…”

Section: Introductionmentioning

confidence: 99%