Epidermolysis bullosa acquisita with combined features of bullous pemphigoid and cicatricial pemphigoid

Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal blisterin g disease associated with autoantibodies against type VII collagen. The cla ssical or mechanobullous: form of EBA is characterized by skin fragility, t rauma-induced blisters and erosions with mild mucous membrane involvement a nd healing with scars. Furthermore, bullous-pemphigoid-like and cicatricial pemphigoid-like features have been described. We report a patient who deve loped a bullous skin disease with upper airway obstruction requiring trache otomy. The diagnosis of EBA was established by immunoblot, showing a band a t 290 kD (collagen VII), and NaCl-split skin immunofluorescence (IgG deposi tion at the floor of the split). This case presented with clinical features of both bullous pemphigoid and cicatricial pemphigoid which to our knowled ge is the first report of such a combination in EBA. The patient also prese nted tracheal involvement that has never been described either.