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Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
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Retrospective Study on Autoimmune Blistering Disease in Paediatric Patients
Yan Ling Kong1, Yen Loo Lim1, Nisha Suyien Chandran2
1Department of Dermatology, National Skin Centre, Singapore, Singapore.
Pediatric Dermatology
|September 23, 2015
Summary
Linear immunoglobulin A disease (LAD) is the most common autoimmune blistering disease in children in Singapore. Treatment with rituximab shows promise for severe childhood pemphigus vulgaris (PV).
Area of Science:
- Pediatric Dermatology
- Autoimmune Blistering Diseases
- Immunopathology
Background:
- Autoimmune blistering diseases (AIBDs) are rare pediatric conditions with unclear prevalence in Singapore.
- This study investigates the clinical and immunopathologic features of childhood AIBDs in Singapore.
Purpose of the Study:
- To determine the prevalence and characteristics of AIBDs in children in Singapore.
- To analyze clinical presentation, triggers, investigations, treatments, and disease course.
Main Methods:
- Retrospective analysis of pediatric AIBD patients (<18 years) diagnosed between 1998-2012 at the National Skin Centre, Singapore.
- Review of clinical and histology databases, including demographic data, presentation, triggers, investigations, treatments, and disease outcomes.
Main Results:
- Twelve pediatric AIBD cases were identified: Linear immunoglobulin A disease (LAD) was most common (41.7%), followed by pemphigus vulgaris (PV), bullous pemphigoid, bullous systemic lupus erythematosus, and pemphigus foliaceus.
- The mean age of onset was 8.7 years. Most patients received steroids and immunosuppressants; intravenous rituximab was effective in a recalcitrant PV case.
- At a mean follow-up of 2.35 years, 33.3% achieved complete remission off therapy, 16.7% in complete remission on therapy, and 33.3% in partial remission.
Conclusions:
- Linear immunoglobulin A disease (LAD) is the most frequent AIBD in children in Singapore, aligning with global literature.
- Dermatitis herpetiformis, common in the West, was not observed in this cohort.
- Intravenous rituximab is a potential option for recalcitrant childhood pemphigus vulgaris (PV), necessitating careful side effect monitoring. Immunohistopathology is crucial for diagnosis and atypical cases.
