CHILDHOOD LINEAR IGA BULLOUS DERMATOSIS W ITH AUTOANTIBODIES TO THE 180 KDA PEMPHIGOID ANTIGEN

Background.- Linear IgA bullous dermatosis (LABD) is an autoimmune sub epidermal blistering disease defined on the basis of direct immunofluo rescence findings. Case report.- An 18 month-old girl suffering from L ABD was studied by indirect immunofluorescence on salt-split skin and by Western blot in an attempt to characterize the involved autoantigen . Direct immunofluorescence showed an exclusive linear IgA deposit at the dermal-epidermal junction. Indirect immunofluorescence revealed ci rculating autoantibodies that reacted with the epidermal side of salt- split skin; they reacted by Western blot with a 180 kDa epidermal anti gen, as in bullous pemphigoid. Conclusion.- This dermatosis fulfilling the clinical features and direct immunofluorescence criteria for chil dhood LABD seems to represent a case of IgA bullous pemphigoid. It fur ther underscores the nosologic heterogeneity of LABD, which probably i ncludes, apart from bullous pemphigoid, epidermolysis bullosa acquisit a and cicatricial pemphigoid.