Mucosal morbidity in patients with epidermolysis bullosa acquisita

Background: Epidermolysis bullosa acquisita is an acquired inflammatory and /or dermolytic subepidermal blistering disease characterized by IgG autoant ibodies to type VII collagen. Four patients with documented epidermolysis b ullosa acquisita were evaluated by a multidisciplinary team of care provide rs (4 dermatologists, an ophthalmologist, a radiologist, a voice and speech specialist, and an otolaryngologist) for 1 to 5 years to characterize muco sal involvement and its complications and response to treatment. Patients w ere evaluated clinically and by slitlamp examinations, endoscopies, compute d tomographic scans, and videofluorographic swallowing studies. Spiral comp uted tomographic scans for virtual endoscopy were used for the nontraumatic evaluation of airways in 2 patients with respiratory tract compromise. Observations: Involvement of 5 or more mucosal sites-mouth, nose, conjuncti va, pharynx, and larynx-was documented in all patients. Complications inclu ded ankyloglossia, periodontal disease, scarring and crusting of nasal muco sa, symblepharon formation, obstruction of nasolacrimal ducts, deformation of the epiglottis, impaired phonation, dysphagia, esophageal strictures, an d supraglottic stenosis requiring emergency tracheostomy. Conclusions: Epidermolysis bullosa acquisita may extensively (or predominan tly) affect mucosal epithelia in a manner resembling cicatricial pemphigoid . Mucosal disease in these patients is often subclinical, can lead to serio us complications, and is best managed using a multidisciplinary approach.