Paraneoplastic pemphigus: Revised diagnostic criteria based on literature analysis

Svoboda, Steven A; Huang, Simo; Liu, Xinyang; Hsu, Sylvia; Motaparthi, Kiran

doi:10.1111/cup.14004

Cited by 18 publications

(14 citation statements)

References 17 publications

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“…Minor criteria, (a) Acantholysis and/or lichenoid interface observed on histopathology, ±necrotic keratinocytes; (b) DIF displaying intercellular and/or basement membrane staining. 6 In the present case, the patient had no obvious oral mucosa involvement, erythema multiforme-like and lichen planus-like lesion. Histopathology did not show keratinocyte necrosis, liquefaction degeneration of basal cell, and dense band-like infiltration of lymphocytes in dermis.…”

Section: Discussionmentioning

confidence: 45%

Pemphigus Herpetiformis-Type Drug Reaction Caused by Programmed Cell Death Protein-1 Inhibitor Treatment

Zhang

Zhang²,

Xie³

et al. 2021

CCID

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Reports of immune-related adverse events caused by programmed cell death protein-1 inhibitor are becoming increasingly frequent. Herein, we report the first case of pemphigus herpetiformis-type drug reaction presented after the treatment of tislelizumab (6 cycles) in a primary non-small cell lung carcinoma patient. A 56-year-old Chinese man was referred to our department for pruritic annulare erythema and blister for two weeks. Histological finding revealed blister formation in the epidermis and eosinophilic infiltration in the blister fluid. Direct immunofluorescence showed intercellular deposition of IgG and C3 within the lower part of epidermis. Serum anti-intercellular antibodies were positive at 1:100 dilution. Based on history and clinicopathological correlation, herpetiformis-type drug-induced pemphigus was diagnosed, which was possibly be induced by tislelizumab. To the best to our knowledge, there is no report of pemphigus herpetiformis-type drug-induced reaction associated with programmed cell death protein-1 inhibitor treatment.

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Section: Discussionmentioning

confidence: 45%

Pemphigus Herpetiformis-Type Drug Reaction Caused by Programmed Cell Death Protein-1 Inhibitor Treatment

Zhang

Zhang²,

Xie³

et al. 2021

CCID

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“…Therefore, PNP was ruled out based on the clinical features, typical histopathological findings, natural disease course, satisfactory response to conventional PV treatment, and a long malignancy‐free follow‐up. However, because more than 95% of cases with PNP present with a preexisting malignancy or develop a concomitant neoplasm soon after the onset of MCPV symptoms, the extended follow‐up of our cases minimises the chance of misdiagnosis 4,25 …”

Section: Discussionmentioning

confidence: 99%

“…However, because more than 95% of cases with PNP present with a preexisting malignancy or develop a concomitant neoplasm soon after the onset of MCPV symptoms, the extended follow‐up of our cases minimises the chance of misdiagnosis. 4 , 25 …”

Section: Discussionmentioning

confidence: 99%

Indirect immunofluorescence on rat bladder epithelium in patients with pemphigus vulgaris with an extended follow‐up

Samadi

Najafi

Naziriyan

et al. 2022

Skin Health and Disease

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Background Indirect immunofluorescence (IIF) on rat bladder epithelium (RBE) has been widely used to detect anti‐plakin antibodies present in paraneoplastic pemphigus (PNP). However, anti‐plakin antibodies have also been found in a group of patients with pemphigus vulgaris (PV). Objectives To assess the reactivity rate of PV sera in IIF using RBE as substrate and the diagnostic usefulness of the aforementioned test. Methods Patients diagnosed with PV presenting to Razi Hospital, Tehran, Iran, were recruited. The patients' demographics, disease severity, and response to the initial treatment were recorded. Sera were collected and tested by IIF on RBE and by desmoglein 3/1 (Dsg 3/1) enzyme‐linked immunosorbent assay. Patients were followed up closely for a mean of 53.9 months for any evidence of malignancy. Results Forty‐six patients were enroled (mean age of 42.9 years old, 31 females). Nine sera (19.6%) showed reactivity in IIF on RBE. Mean serum anti‐Dsg levels did not differ significantly among the two groups with positive and negative IIF results. Negative anti‐Dsg3 was related to a higher positive rate in IIF on RBE. There was no significant correlation between the reactivity of IIF on RBE and patients' demographic, clinical, or serological characteristics. Conclusions IIF on RBE is a sensitive test for detecting antibodies against plakins. However, it has a relatively high false‐positive rate in PV, probably due to the epitope spreading phenomenon. This test should be suggested when there is a clinical or immunohistopathological suspicion of PNP and should be interpreted with caution.

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“…Meeting all three major criteria or two major and both minor criteria fulfill a diagnosis of PNP. Major criteria include (a) mucositis with or without cutaneous involvement, (b) concomitant internal neoplasm, and (c) serologic evidence of anti-plakin antibodies; minor criteria include (a) acantholysis and/or lichenoid interface dermatitis on histopathology and (b) direct immunofluorescence staining, showing intercellular and/or basement membrane staining [37].…”

Section: Paraneoplastic Pemphigusmentioning

confidence: 99%

“…Concurrent treatment with standard corticosteroid therapy, with or without other immunosuppressive agents, is necessary in most cases [30,35]. Rituximab is often used in PNP, although the response to therapy is variable (Figure 8) [37]. Refractory PNP is particularly difficult to treat; various monoclonal antibodies have been used successfully in case reports, including alemtuzumab (anti-CD52 monoclonal antibody) [63].…”

Section: Paraneoplastic Pemphigusmentioning

confidence: 99%

An Updated Review of Pemphigus Diseases

et al. 2021

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Clinicians may encounter a variety of skin conditions that present with vesiculobullous lesions in their everyday practice. Pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus represent the spectrum of autoimmune bullous dermatoses of the pemphigus family. The pemphigus family of diseases is characterized by significant morbidity and mortality. Considering the risks associated with a delayed diagnosis or misdiagnosis and the potential for overlap in clinical features and treatment, evaluation for suspected pemphigus disease often requires thorough clinical assessment and laboratory testing. Diagnosis is focused on individual biopsies for histopathology and direct immunofluorescence. Additional laboratory methods used for diagnosis include indirect immunofluorescence and enzyme-linked immunosorbent assay. Recent advancements, including anti-CD20 therapy, have improved the efficacy and reduced the morbidity of pemphigus treatment. This contribution presents updates on the pathophysiology, clinical features, diagnostic work-up, and medical management of pemphigus. Improved strategies for diagnosis and clinical assessment are reviewed, and newer treatment options are discussed.

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Paraneoplastic pemphigus: Revised diagnostic criteria based on literature analysis

Cited by 18 publications

References 17 publications

Pemphigus Herpetiformis-Type Drug Reaction Caused by Programmed Cell Death Protein-1 Inhibitor Treatment

Pemphigus Herpetiformis-Type Drug Reaction Caused by Programmed Cell Death Protein-1 Inhibitor Treatment

Indirect immunofluorescence on rat bladder epithelium in patients with pemphigus vulgaris with an extended follow‐up

An Updated Review of Pemphigus Diseases

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