IgE bullous disease

We report two patients with the typical picture of bullous pemphigoid who lacked two critical diagnostic immunopathological features of the disease, namely IgG or C3 bound to the epidermal basement membrane and circulating IgG antibodies directed against the basement membrane zone (BMZ). Both patients had dense infiltrates of eosinophils within their skin lesions, as well as markedly elevated serum IgE levels, while immunofluorescent studies with anti-IgE antibody revealed heavy IgE deposition on inflammatory cells within the dermis surrounding the bullae. These cells were confirmed to be eosinophils by means of specific staining with antibody to major basic protein (MBP). We speculate that this 'IgE bullous disease' resulted from IgE-mediated hypersensitivity 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 bilateral above-the-knee amputations of gangrenous legs (Patient 2).