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
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.


CONCLUSIONS
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.
Metry
Denise W
DW
Department of Dermatology, Texas Children's Hospital, Baylor College of Medicine, 6621 Fasnnin Street, MC 3-3315, Houston, TX 77030-2399, USA. dwmetry@texaschildrenshospital.org
Hebert
Adelaide A
AA
Jordon
Robert E
RE
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D002675:Child, Preschool; D003622:Dapsone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D015994:Incidence; D008297:Male; D010392:Pemphigus / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D011239:Prednisolone / Q000627:therapeutic use; D012189:Retrospective Studies
D000893:Anti-Inflammatory Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal; D005938:Glucocorticoids; D006886:Hydroxychloroquine; D003622:Dapsone; D011239:Prednisolone