Bullous pemphigoid (BP) is the most common acquired autoimmune bullous dermatosis, and is extremely rare in children and infants. 1 We report a case of a child with infantile BP (IBP) that was successfully treated with intravenous immunoglobulin (IVIG) therapy.A 4-month-old boy presented with erythematous skin lesions and multiple vesicles on his hands and feet. He had originally been seen by his paediatrician, but as the erythematous lesions and blisters continued to develop, he was referred to us. He was previously healthy, and Nikolsky sign was absent. His medical history was unremarkable. DPT-IPV (combined diphtheria, Bordetella pertussis, tetanus and poliovirus), Haemophilus influenza B and Pneumoccocus immunizations had been given 10 days before his visit to the paediatrician. There was no parental history of skin blistering.Physical examination revealed urticarial plaques with tense bullae and small vesicles on the patient's arms, palms and soles. There were urticarial plaques with erosions on his face, trunk ( Fig. 1a) and limbs (Fig. 1b,c). Mucous membranes were not involved, and the rest of the physical examination was unremarkable.Histopathological examination showed a subepidermal bulla containing eosinophils (Fig. 2a,b). Direct immunofluorescence (DIF) demonstrated linear deposition of IgG (Fig. 2c) and C3 (Fig. 2d) along the dermoepidermal junction. ELISA of the patient's serum demonstrated elevated IgG autoantibodies against the NC16a domain of BP antigen 180 (BP180; also termed BP antigen 2 or type XVII collagen) at 126 (normal range: < 9).The child was diagnosed as having IBP and was initially treated with topical and oral corticosteroids (prednisolone 1 mg/kg/day), but the erythema and blisters continued to spread, so the oral prednisolone dose was increased to 2 mg/kg daily and erythromycin 40 mg/kg daily was started. However, this produced no improvement, and adverse effects (AEs) began to develop, such as moon face and impaired liver function. Therefore, a 1-day course of intravenous immunoglobulin (IVIG) therapy 2 g/kg/day was initiated. Five days after the IVIG therapy, no new lesions had developed. Recurrence of blistering occurred 2 weeks after first IVIG therapy and a second course of IVIG (2 g/kg/day) was given, which resulted in significant improvement. A third and fourth course of IVIG (1 g/kg/day) were given, and the dose of prednisolone was gradually tapered to 0.1 mg/kg/day (Fig. 2e). None of the potentially serious AEs of IVIg, such as anaphylaxis or renal failure, were experienced. During the subsequent 2-year follow-up period, the patient has not had a recurrence. IBP is quite rare. 2 Furthermore, Waisbourd-Zinman et al. compared BP in infancy with childhood BP, and reported significantly higher predisposition for acral involvement in IBP. However, no major differences regarding laboratory results, treatments and prognosis were found.