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Giornale Italiano di Dermatologia e Venereologia 1998 February;133(1):13-21


language: Italian

Linear IgA disease. Immunological observations

Caproni M., Rolfo S., Fabbri P.

Università degli Studi - Firenze, Istituto di Clinica Dermatologica II


Linear IgA bullous dermatosis (LAD) is an acquired, autoimmune, dapsone-responsive, subepidermal blistering disease characterized by linear deposits of IgA at the dermo-epidermal basement membrane zone (BMZ), often with circulating IgA antibodies to the BMZ. It occurs in both adults (adult LAD) and children (chronic bullous disease of childhood). The remission before puberty generally occurs in children, but it is not common in patients with adult LAD. Clinical manifestations may resemble either a bullous pemphigoid-like pattern or a dermatitis herpetiformis-like pattern or characteristically may show a “jewel-like” morphology (clusters and rosettes of blisters). The histopathology of LAD appears similar both to that seen in dermatitis herpetiformis (with neutrophilic microabscesses and fibrin at the papillary tips) and, in some cases, to that of bullous pemphigoid (with a predominance of eosinophils). Indirect immunofluorescence using 1M NaCl split skin demonstrated that LAD sera react both with the epidermal side and with the dermal side. The ultrastructural localization of the IgA deposits has been localized to the lamina lucida, sublamina densa or both, forming a mirror image pattern. Target antigens of the circulating autoantibodies are thought to be a 97 kDa epidermal-associated-lamina lucida antigen, a 285 kDa dermal-associated-sub-lamina densa antigen and a 255 kDa dermal-associated antigen. The 97-kDa protein, the major LAD antigen (LAD-1), which localizes to the epidermal side of the salt split skin, is detected most frequently and is now considered an anchoring filament protein. Furthermore there is an association in both children and adults with HLA-B8, -DR3,-DQw1, -DQw2 . These clinical, histopathological, immunological and immunogenetic differences do not correlate with each other and emphasize the difference and heterogeneity of LAD.

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