Histopathological, cytomorphological and electron microscopic analyses in a case of fogo selvagem are reported. Contradictory to the light microscopical findings, the acantholysis as seen with the electron microscope involves the basal layer but not the subcorneal layers--at least not the most superficial part of the granular layer. A conspicuous disintegration of the tonofilament-desmosome complexes give rise to the concomitant dyskeratosis. This aberrant process, including an association between retracing tonofilaments and defective Odland bodies, results in the terminal stage of monstrous defective and specific keratohyalin. In cytoplasm, target-like structures similar to virions of the herpes virus group were observed. The dynamics of the pathological process is discussed.
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Genetic Association and Differential RNA Expression of Histone (De)Acetylation-Related Genes in Pemphigus Foliaceus-A Possible Epigenetic Effect in the Autoimmune Response.
Life (Basel)
December 2023
Laboratory of Human Molecular Genetics, Department of Genetics, Federal University of Paraná (UFPR), Curitiba 81531-980, Brazil.
Article Synopsis
- Pemphigus foliaceus (PF) is an autoimmune skin disease caused by the production of antibodies against desmoglein-1, with a specific endemic form (EPF) prevalent in Brazil.
- A study examined the genetic relationship between histone (de)acetylation-related genes and EPF, identifying certain genetic variants that increase or decrease susceptibility to the disease.
- Additionally, RNA sequencing revealed altered expression levels of specific genes in CD4 T lymphocytes from untreated EPF patients, suggesting these genes may play a role in immune response and disease pathology.
Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil.
An Bras Dermatol
January 2024
Service of Dermatology, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil; Postgraduate Programa in Science Applied to Adult Health, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil; Department of Internal Medicine, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
Article Synopsis
- Pemphigus is a group of autoimmune skin diseases, with recent trends showing a decrease in endemic pemphigus foliaceus and an increase in pemphigus vulgaris; however, no research has focused on this issue in Minas Gerais, Brazil.
- The study aimed to investigate the epidemiological and clinical profiles of patients diagnosed with pemphigus at a university hospital over a six-month period, involving 122 patients categorized into those with pemphigus foliaceus and those with pemphigus vulgaris.
- Significant differences were found between the two groups in several factors, including age at diagnosis, occupation, living environment, and the number of medications needed for treatment, indicating changes in the disease's landscape over time.
The landscape of the immunoglobulin repertoire in endemic pemphigus foliaceus.
Front Immunol
August 2023
Programa de Pós-Graduação em Genética, Universidade Federal do Paraná, Curitiba, Brazil.
Article Synopsis
- - Pemphigus foliaceus (PF) is an unusual autoimmune skin disease primarily caused by autoreactive B cells, with notable prevalence in Brazil's endemic regions, suggesting environmental factors play a significant role in its development.
- - The study analyzed the B-cell repertoire of PF patients in various states—untreated, under treatment, and in remission—compared to healthy individuals from both endemic and non-endemic areas using advanced sequencing techniques.
- - Results showed significantly lower clonotype diversity in the endemic patients, longer CDR3 sequences, and specific IGHV segment usage linked to active disease, indicating that both environmental influences and disease status affect immune responses.
miRNA implication in the pathogenesis and the outcome of Tunisian endemic pemphigus foliaceus.
Exp Dermatol
July 2023
Autoimmunity, Cancer and Immunogenetics Research Laboratory (LR18SP12), Immunology Department, Habib Bourguiba Hospital, University of Sfax, Sfax, Tunisia.
Article Synopsis
- Pemphigus foliaceus (PF) is an autoimmune skin disease diagnosed via blood and skin tests, with severity linked to anti-Dsg1 antibody levels and unpredictable prognosis.
- A study examined the expression of specific microRNAs (miRNAs) in blood and skin samples from PF patients, finding higher miRNA levels in blood compared to skin samples.
- Key findings indicated that blood miR-21 may serve as a diagnostic marker for untreated patients, while cutaneous miR-155 may predict disease activity, suggesting both could enhance PF diagnosis and monitoring alongside traditional methods.
Patterns of Antinuclear Antibodies in a New Variant of Endemic Pemphigus in El Bagre, Colombia, Colocalizing with Antigens against MIZAP, ARVCF, p0071, and Desmoplakins I and II.
J Appl Lab Med
October 2022
Georgia Dermatopathology Associates, Atlanta, GA, USA.
Article Synopsis
- A new variant of pemphigus foliaceus, named El Bagre-EPF, exhibits a distinctive pattern of antinuclear antibodies (ANAs) that was studied in 57 patients compared to 57 control subjects.
- The research revealed that 24% of El Bagre-EPF patients showed a unique ANA pattern, characterized by specific nuclear staining, which was statistically significantly different from the control group.
- This new ANA pattern was linked with certain proteins (MIZAP, ARVCF, p0071, and desmoplakins I-II) and also showed that 14% of patients exhibited classic homogeneous ANA patterns related to other specific autoantibodies.
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