LINEAR IGA BULLOUS DERMATOSIS OF CHILDHOOD WITH AUTOANTIBODIES TO A 230-KDA EPIDERMAL ANTIGEN

Linear IgA bullous dermatosis (LABD) is an autoimmune, subepidermal di sease defined on the basis of direct immunofluorescence findings. Howe ver, more recent techniques used to study bullous dermatoses suggest t hat LABD may be heterogeneous. A patient with LABD of childhood (chron ic benign disease of childhood, CBDC) was studied by indirect immunofl uorescence on salt-split skin and by Western blot in an attempt to cha racterize the involved autoantigen. This young girl's periorificial (m outh, genitalia), erythematovesicular lesions were diagnosed initially as herpes simplex. Histologic examination revealed eosinophilic spong iosis, suggesting the diagnosis of an autoimmune blistering disease. D irect immunofluorescence showed an exclusive linear IgA deposit at the dermoepidermal junction. Indirect immunofluorescence revealed circula ting IgA autoantibodies that reacted with the epidermal side of salt-s plit skin; these reacted by Western blot with a 230 kDa epidermal anti gen, as in bullous pemphigoid. This case, fulfilling the diagnostic cl inical and direct immunofluorescence criteria for LABD/CBDC, seems to represent IgA bullous pemphigoid. It further underscores the nosologic heterogeneity of LABD, which probably includes, apart from bullous pe mphigoid, epidermolysis bullosa acquisita and cicatricial pemphigoid.