JUVENILE LINEARE IGA-DERMATOSE MOGLICHKEITEN DER DIAGNOSTIK

An 18-month-old girl presented with an eruption of tense blisters on the face, oral mucous membranes, arms and legs arising over the past 3 weeks. Histologically, we found a subepidermal blister formation. Direct immunofluorescence demonstrated a linear deposition of IgA at the basement membrane zone. The indirect immunofluorescence with NaCl-separated human skin as a substrate revealed circulating IgA serum antibodies binding to the roof of the artificial blister. By immunoblotting of epidermal and dermal extracts, the antibodies recognized an epidermal protein of 97 kD. Three years later, the disease still requires oral treatment with dapsone and low-dose corticosteroids to suppress new blister formation.