Head and neck manifestations of epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita (EBA) is a rare, chronic, acquired bullous autoimmune dermatosis. It is characterized by the formation of IgG autoanti bodies against type VII procollagen of anchoring fibrils with subepidermal formation of bullous lesions and consequent scarring. The epidemiology of t his disease and its characteristic clinical findings cannot be completely s urveyed at present due to the limited number of available publications. In general, bullous lesions form on the entire integument and can also involve mucosa. The development of scar-related adhesions on the mucosa of the upp er airways and esophagus can lead to serious complications that are difficu lt to treat. We report our experience in managing a 44-year-old male patien t in whom the diagnosis of EBA was established in 1993 on the basis of mult iple persistent bullous lesions involving the eye, nose, skin and oral, pha ryngeal and laryngeal mucosa. After failing previous medical and surgical t herapies, interdisciplinary management resulted in the control of active le sions with extracorporal phototherapy and cyclosporin A.