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12:23
Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Nonendemic pemphigus foliaceus in children
Denise W Metry1, Adelaide A Hebert, Robert E Jordon
1Department of Dermatology, Texas Children's Hospital, Baylor College of Medicine, 6621 Fasnnin Street, MC 3-3315, Houston, TX 77030-2399, USA. dwmetry@texaschildrenshospital.org
Journal of the American Academy of Dermatology
|February 28, 2002
Summary
Sporadic pemphigus foliaceus in children is typically mild and short-lived, though diagnosis can be challenging. New tests and treatments like hydroxychloroquine offer hope for managing this rare autoimmune blistering disease.
Area of Science:
- Dermatology
- Autoimmune Diseases
- Pediatrics
Background:
- Pemphigus foliaceus is a rare autoimmune blistering skin disease with endemic (fogo selvagem) and sporadic forms.
- The sporadic form typically affects middle-aged and elderly individuals, rarely occurring in children.
Observation:
- This study presents a case of sporadic pemphigus foliaceus in a 3-year-old boy and reviews 28 previous pediatric cases.
- Unique skin lesion patterns like "arcuate," "circinate," or "polycyclic" were observed in pediatric cases.
Findings:
- Sporadic pemphigus foliaceus in children generally follows a benign, short-term course compared to pemphigus vulgaris.
- Diagnosis can be challenging using routine histology or immunopathology.
Implications:
- Antigen-specific tests (e.g., ELISA for anti-desmoglein 1 autoantibodies) can expedite diagnosis.
- Hydroxychloroquine is a potential treatment for children with photodistributed lesions.
- Further long-term outcome studies are needed to guide treatment decisions in pediatric cases.
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