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).