Paraneoplastic Dermatoses: A Brief General Review and an Extensive Analysis of Paraneoplastic Pemphigus and Paraneoplastic Dermatomyositis

Didona, Dario; Fania, Luca; Didona, Biagio; Eming, Rüdiger; Hertl, Michael; Zenzo, Giovanni Di

doi:10.3390/ijms21062178

Cited by 54 publications

(68 citation statements)

References 63 publications

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“…An explanation could be that paraneoplastic skin manifestations of ovarian cancer were wrongly classified as CLE. 43 However, paraneoplastic skin manifestations of ovarian cancer are quite rare, and even if it was the cause, it would be suspected that the cancer diagnoses were registered within the first year of follow-up due to medical scrutiny. Looking at our data (not shown), zero cases of ovarian cancer were registered during the first year of follow-up.…”

Section: Discussionmentioning

confidence: 99%

Increased cancer risk in patients with cutaneous lupus erythematosus and systemic lupus erythematosus compared with the general population: A Danish nationwide cohort study

Westermann

Zobbe

Cordtz

et al. 2021

Lupus

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Objectives To investigate if patients with cutaneous lupus erythematosus (CLE) or systemic lupus erythematosus (SLE) have an increased risk of cancer compared with the general population, and furthermore to identify specific cancer types associated with increased risk. Methods This is an observational cohort study of 5310 patients with CLE or SLE identified in the Danish National Patient Register from 1 January 1995 to 31 December 2014. The cohort was followed up for cancer by linkage to the Danish Cancer Registry. Based on the age, sex, and calendar specific cancer rates of the general population of Denmark, standardised incidence ratios (SIRs) were calculated. Results The patients with CLE or SLE were followed for 40.724 person-years, each group’s average duration of follow-up being 6.9 and 8.1 years. The SIR for overall cancer (except non-melanoma skin cancer (NMSC)) was increased in patients with CLE 1.35 (95%CI 1.15 to 1.58) and patients with SLE 1.45 (95%CI 1.30 to 1.62). Both groups had high risks of hematological – including a 3–4-fold increased risk of non-Hodgkin lymphoma –, pancreatic, and lung cancers. Several cancers associated with oncogenic viruses as liver and tongue/mouth/pharynx were increased in the SLE group, while the risk of ovarian cancer was increased 2–4-fold only in the CLE group. Conclusion The overall risk of cancer was significantly increased in both patients with CLE and SLE. SIRs for hematological, pancreatic and lung cancers were elevated in both groups. Extra awareness of cancer in patients with SLE and patients with CLE should be considered.

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Section: Discussionmentioning

confidence: 99%

Increased cancer risk in patients with cutaneous lupus erythematosus and systemic lupus erythematosus compared with the general population: A Danish nationwide cohort study

Westermann

Zobbe

Cordtz

et al. 2021

Lupus

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“…Besides, the scalp was also reported to be involved by scaling or erythema. [17][18][19][20] Dermal mucin accumulation, basal keratinocytes-related vacuolar changes, and dermal lymphatic infiltration that is usually mild to moderate are the main histological manifestations of the disease, in addition to some dermal sclerosis. 21 In another context, poikiloderma (telangiectasias, and dyspigmentations, together with epidermal atrophic changes to the same affected regions) is usually the manifestation of chronic dermatomyositis.…”

Section: Dermatomyositismentioning

confidence: 99%

Skin manifestations of autoimmune diseases in the pediatrics

Alyamani

Alsuwayq

Bahatheq³

et al. 2021

Int J Community Med Public Health

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Autoimmune diseases during childhood are common and are usually observed to affect multiple systems. Among many pediatric autoimmune diseases, several cutaneous manifestations were reported in the literature, and the adequate examination and detection of these manifestations might significantly enhance the chances of establishing an adequate diagnosis of the underlying diseases and conditions because the affected patients usually present with typical symptoms. Establishing a proper diagnosis can enhance the prognosis of the affected patients and might prevent the development of serious complications and other adverse events. Besides, it was also previously indicated that establishing an early diagnosis will furtherly help with deciding the most appropriate interventions, which will also enhance the prognosis of the pediatric population. In this literature review, we have discussed the cutaneous manifestations of several autoimmune diseases that might affect the pediatric population. We have majorly discussed the conditions that are associated with systemic lupus, juvenile idiopathic arthritis, dermatomyositis, juvenile-onset systemic sclerosis and morphea. The cutaneous manifestations of these conditions are hugely variable and usually need careful examination and adequate differential diagnosis to establish the best management modality. Besides, some manifestations might gradually develop which might even make the diagnostic approaches even more difficult. Accordingly, clinicians should provide full and thorough examination and diagnosis of the suspected patients because detecting such cases might significantly enhance the prognosis and lead to adequate treatment.

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“…PNP is also known as paraneoplastic autoimmune multiorgan syndrome (PAMS) because of its varied mucocutaneous and systemic deleterious effects. Due to the variable disease characteristics and lack of formally accepted diagnostic criteria, PNP remains a diagnostic challenge for clinicians 1‐5 . The original diagnostic criteria proposed by Anhalt et al, who first described PNP in 1990, are based on five key clinical, histopathologic, and immunologic features (Table 1).…”

Section: Introductionmentioning

confidence: 99%

“…While classic histopathologic features of PNP consist of acantholysis and lichenoid interface dermatitis, both findings are not always observed together 1,2,5 . Additionally, newer techniques for identifying plakin proteins such as immunoblot and enzyme‐linked immunosorbent assay (ELISA) have been developed as alternatives to immunoprecipitation, the historic gold standard 2,4,5,8 . Consequently, reevaluating the current diagnostic criteria for PNP is warranted.…”

Section: Introductionmentioning

confidence: 99%

“…Due to the variable disease characteristics and lack of formally accepted diagnostic criteria, PNP remains a diagnostic challenge for clinicians. [1][2][3][4][5] The original diagnostic criteria proposed by Anhalt et al, who first described PNP in 1990, are based on five key clinical, histopathologic, and immunologic features (Table 1). 6 Subsequent attempts at revising these diagnostic guidelines have been made.…”

Section: Introductionmentioning

confidence: 99%

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Paraneoplastic pemphigus: Revised diagnostic criteria based on literature analysis

et al. 2021

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Background Paraneoplastic pemphigus (PNP) is a rare autoimmune bullous disease classically associated with an underlying neoplasm. The heterogeneous clinical and histopathologic features of the disease make diagnosis challenging for clinicians. There are no formally accepted diagnostic criteria, and newer techniques for identifying antibodies directed against plakin proteins have largely replaced immunoprecipitation, the historic gold standard. Methods An analysis of 265 published cases of PNP was performed. The clinical, histopathologic, and immunologic features of PNP were assessed. Results Based on this review, we modified previous diagnostic criteria to capture 89.4% of PNP cases compared to 71.2% of cases captured by the most commonly referenced criteria devised by Camisa and Helm (p‐value < 0.01, z‐test; 95% CI [10.2, 33.6]). Conclusion These revised diagnostic criteria address the variable clinical, histopathologic, and biochemical features of PNP, allowing physicians to have greater confidence in diagnosis of this rare and often fatal disease. The revised criteria include three major criteria and two minor criteria, whereby meeting either all three major criteria or two major and both minor criteria would fulfill a diagnosis of paraneoplastic pemphigus. The major criteria include (a) mucous membrane lesions with or without cutaneous involvement, (b) concomitant internal neoplasm, and (b) serologic evidence of anti‐plakin antibodies. The minor criteria include (a) acantholysis and/or lichenoid interface dermatitis on histopathology and (b) direct immunofluorescence staining showing intercellular and/or basement membrane staining.

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Paraneoplastic Dermatoses: A Brief General Review and an Extensive Analysis of Paraneoplastic Pemphigus and Paraneoplastic Dermatomyositis

Cited by 54 publications

References 63 publications

Increased cancer risk in patients with cutaneous lupus erythematosus and systemic lupus erythematosus compared with the general population: A Danish nationwide cohort study

Increased cancer risk in patients with cutaneous lupus erythematosus and systemic lupus erythematosus compared with the general population: A Danish nationwide cohort study

Skin manifestations of autoimmune diseases in the pediatrics

Paraneoplastic pemphigus: Revised diagnostic criteria based on literature analysis

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