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Endemic pemphigus foliaceus in Venezuela: report of two children
Francisco González1, Ana Maria Sáenz, Antonietta Cirocco
1Department of Dermatology, Hospital Universitario de Caracas, Luis Razetti School of Medicine, Central University of Venezuela, Caracas, Venezuela.
Insights
Endemic pemphigus foliaceous, a skin disease, was diagnosed in two Yanomami children in Venezuela. A specific human leukocyte antigen (HLA) subtype was identified, sharing similarities with those found in Brazil.
Area of Science:
- Dermatology
- Immunogenetics
- Clinical Medicine
Background:
- Endemic pemphigus foliaceous (EPF) is a chronic autoimmune blistering disease.
- EPF is historically documented in Brazil and other parts of South America.
- The genetic associations, particularly human leukocyte antigen (HLA) class II alleles, are well-studied in Brazilian populations.
Observation:
- Two indigenous Yanomami children from the Venezuelan Amazon presented with erythroderma.
- Clinical, histological, and immunofluorescence examinations confirmed the diagnosis of endemic pemphigus foliaceous.
- Genetic analysis revealed the human leukocyte antigen (HLA) DRB1*04:011 subtype in both patients.
Findings:
- The identified HLA-DRB1*04:011 subtype has not been previously linked to endemic pemphigus foliaceous.
- This subtype shares a common epitope with previously implicated HLA-DRB1 alleles in Brazilian EPF patients.
- This represents the first documented cases of endemic pemphigus foliaceous in Venezuela.
Implications:
- The findings expand the known geographical distribution of endemic pemphigus foliaceous to Venezuela.
- The shared epitope suggests a common immunogenetic mechanism underlying EPF across different South American populations.
- Further research is warranted to understand the specific role of HLA-DRB1*04:011 in EPF pathogenesis and its prevalence in the region.
Abstract:
Two native Yanomami children from the Venezuelan Amazonia with erythroderma were hospitalized on our service. Clinical, histologic, and immunofluorescence studies diagnosed endemic pemphigus foliaceous. Human leukocyte antigen class II showed DRB1*04 subtype *0411, which has not been previously associated with this disease. However, it shares a common epitope with all the human leukocyte antigen DRB1 alleles that have been involved in this disease among Brazilian populations. Although this condition is endemic in Brazil, our patients are the first two reported in Venezuela.
