[Epidermolysis bullosa acquisita--manifestations in otorhinolaryngology]

Epidermolysis bullosa acquisita is a rare acquired mechanobullous disorder which in contrast to the other types of epidermolysis bullosa is not hereditary. Skin lesions and, for the first time, lesions of the mucous membranes of the upper respiratory tract and oesophagus were studied by light and electron microscopic and immunofluorescence and immunoelectron microscopic methods. Immunofluorescence studies demonstrated linear IgG and complement deposits along the basement membrane zone. Electron microscopy of skin and mucosa revealed a dermolytic subbasal-lamina type of blister formation, diminished and rudimentary anchoring fibrils, and a zone of amorphous material beneath the basal lamina (lamina densa). Immunoelectron microscopic studies demonstrated the deposition of immunoglobulins in the amorphous zone beneath the basal lamina along the dermal-epidermal junction. The results are discussed in terms of current immunological findings.