Abstract: 【Abstract】 A 9-year-old boy presented with pruritic erythema and erosions of the scalp for 1 month, which spread all over the body for 1 week. Histopathological examination of the skin lesions showed blisters forming under the granular layer of the epidermis, a large number of acantholytic cells in the blisters, and perivascular infiltration of lymphocytes and eosinophils in the superficial dermis. Direct immunofluorescence study showed reticular deposition of IgG and complement C3 between epidermal cells, and negative staining for IgM and IgA. Enzyme-linked immunosorbent assay showed the presence of serum anti-Dsg1 antibody （157.00 U/ml）. The patient was diagnosed with pemphigus foliaceus. After admission, the patient showed poor response to the treatment with prednisone at a dose of 40 mg/d. After the treatment with low-dose rituximab （100 mg per week, 4 weeks） combined with prednisone （20 mg/d）, her condition was well controlled. The dosage of prednisone gradually decreased to 7.5 mg/d for maintenance treatment, and no recurrence was observed during 24-month follow-up.

Key words: Pemphigus, Child, Prednisone, Pemphigus foliaceus, Rituximab