Epidemiology of Pemphigus

Kridin, Khalaf; Schmidt, Enno

doi:10.1016/j.xjidi.2021.100004

Cited by 60 publications

(75 citation statements)

References 166 publications

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“…These data join the epidemiological studies conducted in Iran, Turkey, 26 South Africa, 27 Kuwait, 28 China, 29 and most European countries. 30 The gender specific auto immunity can be explained by hormonal, immunological, microbiomial, and genetic theories (specific genes encoded on the X chromosome). Sex hormones (oestrogens, progresterons) are reported to affect the susceptibility to autoimmune disease with a severe activity disease during pregnancy and relapses in the postpartum period while testosterone has a protective effect on the immune system.…”

Section: Discussionmentioning

confidence: 99%

Epidemiology of Pemphigus: A Single Center Experience in Morocco

Hadadi¹,

Mezni²,

Senouci³

2022

Int J Dermatol Venereol

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Objective: Pemphigus is a life threatening autoimmune bullous disease which involves the skin and mucous membranes of the stratified squamous epithelium. The global distribution of Pemphigus varies according to genetic, ethnic, socioeconomic, and cultural backgrounds. The purpose of our study is to evaluate the epidemiological features of pemphigus a single center in Morocco and compare our results with those reported elsewhere. Methods: A retrospective analysis was conducted of 302 pemphigus patients seen between 1990 and 2020 in the Dermatology Department of Ibn Sina Hospital (Rabat, Morocco). We further collected all the Moroccan scientific researches published by now to compare. Results: The average annual incidence was 0.32/100,000 inhabitants. The incidence doubled to 0.72 in 2020. The most common variant was pemphigus vulgaris (125 cases) followed by pemphigus erythematosus (99 cases), pemphigus foliaceous (40 cases), and vegetans (27 cases). The female to male ratio was 0.75, the average age at onset was 53years old and the mean duration of the disease before diagnosis was 13.36months. Conclusion: This study joins the main characteristics of pemphigus in the Maghreb and around the world (pemphigus vulgaris most frequent subtype). In 2020, an epidemiological peak occurred during the coronavirus disease 2019 pandemic; probably related to stress and delayed time consultation for fear of contracting the severe acute respiratory syndrome coronavirus 2.

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Section: Discussionmentioning

confidence: 99%

Epidemiology of Pemphigus: A Single Center Experience in Morocco

Hadadi¹,

Mezni²,

Senouci³

2022

Int J Dermatol Venereol

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“…Pemphigus is potentially life‐threatening, primarily due to secondary infections. Systemic corticosteroids (CS) have dramatically improved the prognosis, reducing mortality to < 10% 1,4,5 . CS rapidly improve pemphigus symptoms but must be administered at high daily doses (e.g.…”

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confidence: 99%

Treatment of pemphigus vulgaris and foliaceus with efgartigimod, a neonatal Fc receptor inhibitor: a phase II multicentre, open‐label feasibility trial*

et al. 2021

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Summary Background Pemphigus vulgaris and pemphigus foliaceus are potentially life‐threatening autoimmune disorders triggered by IgG autoantibodies against mucosal and epidermal desmogleins. There is an unmet need for fast‐acting drugs that enable patients to achieve early sustained remission with reduced corticosteroid reliance. Objectives To investigate efgartigimod, an engineered Fc fragment that inhibits the activity of the neonatal Fc receptor, thereby reducing serum IgG levels, for treating pemphigus. Methods Thirty‐four patients with mild‐to‐moderate pemphigus vulgaris or foliaceus were enrolled in an open‐label phase II adaptive trial. In sequential cohorts, efgartigimod was dosed at 10 or 25 mg kg−1 intravenously with various dosing frequencies, as monotherapy or as add‐on therapy to low‐dose oral prednisone. Safety endpoints comprised the primary outcome. The study is registered at ClinicalTrials.gov (identifier NCT03334058). Results Adverse events were mostly mild and were reported by 16 of 19 (84%) patients receiving efgartigimod 10 mg kg−1 and 13 of 15 (87%) patients receiving 25 mg kg−1, with similar adverse event profiles between dose groups. A major decrease in serum total IgG and anti‐desmoglein autoantibodies was observed and correlated with improved Pemphigus Disease Area Index scores. Efgartigimod, as monotherapy or combined with prednisone, demonstrated early disease control in 28 of 31 (90%) patients after a median of 17 days. Optimized, prolonged treatment with efgartigimod in combination with a median dose of prednisone 0·26 mg kg−1 per day (range 0·06–0·48) led to complete clinical remission in 14 of 22 (64%) patients within 2–41 weeks. Conclusions Efgartigimod was well tolerated and exhibited an early effect on disease activity and outcome parameters, providing support for further evaluation as a therapy for pemphigus.

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“…PF is less common worldwide than PV [ 2 , 3 ]. However, an endemic form of PF (EPF), also known as fogo selvagem (meaning wild fire, in Portuguese), occurs in Central-Western Brazil and neighboring countries.…”

Section: Introductionmentioning

confidence: 99%

“…PV may be induced or triggered by drugs [ 6 , 7 ]. Various other potential triggers, including ultraviolet and ionizing radiation, vaccines, dietary factors, hormonal disorders, stress, and others, have been discussed [ 3 , 8 , 9 ]. In EPF, epidemiological studies indicate that continued exposure to hematophagous insect bites may be a factor triggering the disease [ 4 , 10 , 11 ].…”

Section: Introductionmentioning

confidence: 99%

Genetic Associations and Differential mRNA Expression Levels of Host Genes Suggest a Viral Trigger for Endemic Pemphigus Foliaceus

Bumiller-Bini

Kohler

Augusto

et al. 2022

Viruses

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The long search for the environmental trigger of the endemic pemphigus foliaceus (EPF, fogo selvagem) has not yet resulted in any tangible findings. Here, we searched for genetic associations and the differential expression of host genes involved in early viral infections and innate antiviral defense. Genetic variants could alter the structure, expression sites, or levels of the gene products, impacting their functions. By analyzing 3063 variants of 166 candidate genes in 227 EPF patients and 194 controls, we found 12 variants within 11 genes associated with differential susceptibility (p < 0.005) to EPF. The products of genes TRIM5, TPCN2, EIF4E, EIF4E3, NUP37, NUP50, NUP88, TPR, USP15, IRF8, and JAK1 are involved in different mechanisms of viral control, for example, the regulation of viral entry into the host cell or recognition of viral nucleic acids and proteins. Only two of nine variants were also associated in an independent German cohort of sporadic PF (75 patients, 150 controls), aligning with our hypothesis that antiviral host genes play a major role in EPF due to a specific virus–human interaction in the endemic region. Moreover, CCL5, P4HB, and APOBEC3G mRNA levels were increased (p < 0.001) in CD4+ T lymphocytes of EPF patients. Because there is limited or no evidence that these genes are involved in autoimmunity, their crucial role in antiviral responses and the associations that we observed support the hypothesis of a viral trigger for EPF, presumably a still unnoticed flavivirus. This work opens new frontiers in searching for the trigger of EPF, with the potential to advance translational research that aims for disease prevention and treatment.

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Epidemiology of Pemphigus

Cited by 60 publications

References 166 publications

Epidemiology of Pemphigus: A Single Center Experience in Morocco

Epidemiology of Pemphigus: A Single Center Experience in Morocco

Treatment of pemphigus vulgaris and foliaceus with efgartigimod, a neonatal Fc receptor inhibitor: a phase II multicentre, open‐label feasibility trial*

Genetic Associations and Differential mRNA Expression Levels of Host Genes Suggest a Viral Trigger for Endemic Pemphigus Foliaceus

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