A Case of Linear IgA Bullous Dermatosis of Childhood / 대한피부과학회지

ABSTRACT

Linear IgA bullous dermatoses(LABD) occurring in childhood is considered to be a childhood counterpart of LABD,rather than chronic bullous dermatosis of childhood. This disease characteristically involves the perioral area,lower trunk,pelvic region and the lower extremities. Immunopathologically,it is characterized by the linear deposits of IgA at the dermoepidermal junction of the perilesional skin. A 20-month-old female infant presented with multiple pruritic tense bullae on the face, lower trunk, both extremities and pelvic region of 2 weeks duration. She had no oral lesions or any specific signs or symptoms referable to the disease of the gastrointestinal tract. A skin biopsy revealed subepidermal blisters infiltrated with many neutrophils and a few eosinophils. Direct immunofluorescence demonstrated linear deposition of IgA at the dermoepidermal junction. An indirect immunofluorescence study using NaC1-split skin as the substrate revealed fluorescence for IgA in a linear pattern on the roof of the split at a titer of 140. The clinical diagnosis of LABD of childhood was confirmed and we started with a combined remedy of dapsone and corticosteroids; 10 weeks later almost all of the lesions had disappeared.