Clinical and immunopathological spectrum of paraneoplastic pemphigus

Zimmermann, Jutta; Bahmer, Friedrich A.; Rosé, Christian; Zillikens, Detlef; Schmidt, Enno

doi:10.1111/j.1610-0387.2010.07380.x

Cited by 68 publications

(106 citation statements)

References 53 publications

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“…varied, typically begins with a florid, painful eruption of mucous membrane lesions, which may involve the oropharynx, nasopharynx, tongue, vermillion of the lips (Figure 1), conjunctiva, anogenital region (Figure 2), or esophagus [1,4,10,11]. Oral mucosal lesions were usually more extensive than that of pemphigus vulgaris or erythema multiforme.…”

Section: Histopathologymentioning

confidence: 99%

“…The onset of cutaneous lesions has a variable delay after the appearance of mucous membrane lesions, ranging from days to months [1,2,4]. Cutaneous features of PNP are polymorphic, including vesicles, blisters, erosions, patches, papules and plaques.…”

Section: Cutaneous Lesionsmentioning

confidence: 99%

“…PNP is an autoimmune syndrome mostly seen in association with hematologic neoplasms including Castleman's disease [5,8, [82], and centroblastic lymphoma [1]. Castleman's disease associated with paraneoplastic pemphigus is misdiagnosed frequently and easily in clinical practices.…”

Section: Associated Tumorsmentioning

confidence: 99%

“…Paraneoplastic Pemphigus (PNP) is a debilitating and lethal adult and pediatric autoimmune blistering skin disease that occurs in the setting of a known or occult neoplasm especially lymphoproliferative neoplasms [1]. In 1990, Anhalt, et al [2] described five atypical pemphigus cases which were associated with lymphoproliferative disease.…”

Section: Introductionmentioning

confidence: 99%

“…More than 260 case reports of PNP have been documented since that time [1,4,5] and about 20 of the reported patients were children or adolescents. Yet PNP may be more common than is generally believed given that serum detection of autoantibodies is only performed by a handful of laboratories.…”

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confidence: 99%

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Paraneoplastic Pemphigus

Zhong-Shuai¹,

Li²

2017

J Dermatol Res Ther

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Paraneoplastic Pemphigus (PNP) is an autoimmune-related acquired bullous disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. Suprabasal acantholysis and clefts with scattered necrotic keratinocytes are the unique histopathological features. PNP patient sera recognize multiple antigens, which have been identified as the plakin protein family. Non-Hodgkin's lymphoma, Castleman's disease, thymoma, follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasms in PNP. The treatment of PNP to date has been rather disappointing. For patients with Castleman's disease, the early detection and removal of the tumor are critical for the treatment of PNP.

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Section: Histopathologymentioning

confidence: 99%

Section: Cutaneous Lesionsmentioning

confidence: 99%

Section: Associated Tumorsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

mentioning

confidence: 99%

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Paraneoplastic Pemphigus

Zhong-Shuai¹,

Li²

2017

J Dermatol Res Ther

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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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