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Updated: Jun 9, 2026
10:27
Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
Published on: December 15, 2011
Pemphigus herpetiformis in childhood
Isabela B Duarte1, Ivander Bastazini, Jaison A Barreto
1Department of Dermatology, Lauro de Souza Lima Institute, Bauru, São Paulo, Brazil. isabeladuarte@gmail.com
Pediatric Dermatology
|September 3, 2010
Summary
Pemphigus herpetiformis (PH) is a rare autoimmune blistering disease. This case highlights a childhood presentation and effective treatment with azathioprine and corticosteroids.
Area of Science:
- Dermatology
- Immunodermatology
- Pediatric Dermatology
Background:
- Pemphigus herpetiformis (PH) is a rare variant of pemphigus.
- It clinically mimics dermatitis herpetiformis but has distinct immunopathological findings.
Observation:
- A 5-year-old female presented with symptoms resembling dermatitis herpetiformis.
- Skin biopsy revealed subcorneal blisters with neutrophils and eosinophils, and rare acantholytic cells.
- Direct immunofluorescence showed intercellular IgG and C3 deposits, confirming pemphigus.
Findings:
- The patient's lesions were refractory to dapsone and systemic corticosteroids.
- Complete remission was achieved using azathioprine combined with high-dose systemic corticosteroids (prednisone).
Implications:
- This report emphasizes the occurrence of pemphigus herpetiformis in childhood.
- It underscores the importance of accurate diagnosis and highlights effective therapeutic strategies for pediatric PH.
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