ABSTRACT
Bullous pemphigoid (BP) is a very rare acquired immunbullous disease in children and infants. We report the case of a 15-month-old boy referred to our service with multiple tense bullae located predominantly on the trunk, neck and proximal portion of the limbs. Palms, soles and oral mucosa were also affected. Histopathologic and immunopathologic features were characterized with bullous pemphigoid. The patient responded well to systemic steroids, with improvement of his condition. After a 10-year follow up the patient was no longer suffering from the disease.
Subject(s)
Pemphigoid, Bullous/pathology , Skin/pathology , Child , Child, Preschool , Fluorescent Antibody Technique, Direct , Follow-Up Studies , Humans , Immunoglobulin G/immunology , Infant , Male , Skin/immunology , Time FactorsABSTRACT
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.
