Immunobullous diseases in Thai children: report of 24 cases.

ABSTRACT

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.