IGE BULLOUS DISEASE

We report two patients with the typical clinical picture of bullous pe mphigoid who lacked two critical diagnostic immunopathological feature s of the disease, namely IgG or C3 bound to the epidermal basement mem brane and circulating IgG antibodies directed against the basement mem brane zone (BMZ).(1) Both patients had dense infiltrates of eosinophil s within their skin lesions, as well as markedly elevated serum IgE le vels, while immunofluorescent studies with anti-IgE antibody revealed heavy IgE deposition on inflammatory cells within the dermis surroundi ng the bullae. These cells were confirmed to be eosinophils by means o f specific staining with antibody to major basic protein (MBP). We spe culate that this 'IgE bullous disease' resulted from IgE-mediated hype rsensitivity induced by focal infection, both patients initially being helped by antibiotics. However, dramatic clearing of bullae was seen following surgical removal of a battery implant (Patient 1), and bilat eral above-the-knee amputations of gangrenous legs (Patient 2).