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Immunobullous diseases in Thai children: report of 24 cases
Srisupalak Singalavanija1, Wanida Limpongsanurak
1Dermatology Division, Queen Sirikit National Institute of Child Health, Bangkok 10400, Thailand.
Insights
Pediatric acquired immunobullous diseases, though rare, show good response to therapies like dapsone and corticosteroids. This study details clinical features, treatment, and outcomes in Thai children.
Area of Science:
- Pediatric Dermatology
- Immunobullous Diseases
- Autoimmune Blistering Disorders
Background:
- Acquired immunobullous diseases in children are uncommon and present diagnostic challenges.
- Distinguishing these rare conditions clinically requires careful evaluation.
Purpose of the Study:
- To investigate the clinical characteristics of pediatric immunobullous diseases.
- To analyze immunopathologic findings, treatment strategies, and outcomes in Thai children.
- To provide insights into the management of rare blistering disorders in pediatric populations.
Main Methods:
- Retrospective review of 24 pediatric cases (under 18 years) diagnosed between 1983-2000.
- Diagnosis confirmed by clinical presentation, histopathology, and direct immunofluorescence studies.
- Analysis of patient demographics, disease subtypes, treatment regimens, and clinical responses.
Main Results:
- The study identified 18 cases of chronic bullous diseases of childhood (CBDC), 4 of bullous pemphigoid (BP), and 2 of pemphigus vulgaris (PV).
- CBDC and BP showed mean onset ages of 4 and 2 years, respectively, with equal gender distribution for both.
- Most CBDC and all BP cases responded well to dapsone and/or corticosteroid therapy; pemphigus cases showed variable responses to treatment.
Conclusions:
- Immunobullous diseases are exceptionally rare in pediatric patients.
- Effective treatment outcomes were observed with corticosteroid and/or dapsone therapies for these conditions.
- Early diagnosis and appropriate management are crucial for favorable prognoses in pediatric immunobullous diseases.
Background:
Acquired immunobullous diseases in children are very rare and difficult to distinguish clinically.
Objective:
To study the clinical manifestations, immunopathologic features, treatment and outcome of immunobullous diseases in Thai children.
Material And Method:
The authors reviewed 24 cases of immunobullous diseases in children under 18 years at Queen Sirikit National Institute of Child Health from 1983 to 2000. Diagnosis of all cases was made by clinical presentations of chronic blistering diseases and confirmed by histopathology and immunofluorescent studies.
Results:
There were 18 cases of chronic bullous diseases of childhood (CBDC), 4 cases of bullous pemphigoid (BP) and 2 cases of pemphigus vulgaris (PV). The mean age of onset of CBDC and BP were 4 years and 2 years respectively. There was an equal male to female ratio in both CBDC and BP. Both cases of pemphigus (neonate and 4 years old) were female. Most CBDC patients (18 cases) responded well to dapsone therapy although 2 cases had to be treated with prednisolone simultaneously. All cases with BP were treated successfully with prednisolone and dapsone. Neonatal pemphigus was treated symptomatically without steroid therapy. The second case of oral pemphigus was controlled with low dose prednisolone.
Conclusion:
Immunobullous diseases are very rare in children. All patients improved with corticosteroid and/or dapsone therapy.
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