Nonendemic pemphigus foliaceus in children

Background: Pemphigus foliaceus is a cutaneous, autoimmune, blistering disease comprising two major categories: endemic and sporadic. The endemic form, also known as fogo selvagem, primarily affects children and young adults in rural Brazil. In contrast, the sporadic form of pemphigus foliaceus is generally a disease of the middle-aged and elderly. Objective and methods: Because the sporadic form of pemphigus foliaceus rarely affects children, information specific to this unique group is lacking. We describe a 3-year-old boy with the disease and retrospectively review data from 28 past cases. Results: In comparison to pediatric cases of pemphigus vulgaris, sporadic pemphigus foliaceus in children tends to follow a generally benign course of relatively short duration. However, long-term outcome studies are lacking. A pattern of skin lesions described as "arcuate," "circinate," or "polycyclic" appears to be a unique and specific presentation of this disease in children. Occasionally; as in our case, the diagnosis may prove difficult to establish by using routine histology or immunopathology. Conclusion: The commercial availability of antigen-specific techniques such as enzyme-linked immunosorbent assay for serum desmoglein 1 autoantibody should eliminate delay in diagnosis. Hydroxychloroquine may be another treatment option for those children with photodistributed lesions. Further experience and long-term outcome studies in children are needed to determine whether some medication side effects may outweigh the risks from the disease itself.