Mucosal morbidity in patients with epidermolysis bullosa acquisita

Markham C. Luke, Thomas N. Darling, Roger Hsu, Ronald M. Summers, Janine A. Smith, Beth I. Solomon, Giovana R. Thomas, Kim B. Yancey

Research output: Contribution to journalArticlepeer-review

77 Scopus citations


Background: Epidermolysis bullosa acquisita is an acquired inflammatory and/or dermolytic subepidermal blistering disease characterized by IgG autoantibodies to type VII collagen. Four patients with documented epidermolysis bullosa acquisita were evaluated by a multidisciplinary team of care providers (4 dermatologists, an ophthalmologist, a radiologist, a voice and speech specialist, and an otolaryngologist) for 1 to 5 years to characterize mucosal involvement and its complications and response to treatment. Patients were evaluated clinically and by slitlamp examinations, endoscopies, computed tomographic scans, and videofluorographic swallowing studies. Spiral computed 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, conjunctiva, pharynx, and larynx-was documented in all patients. Complications included ankyloglossia, periodontal disease, scarring and crusting of nasal mucosa, symblepharon formation, obstruction of nasolacrimal ducts, deformation of the epiglottis, impaired phonation, dysphagia, esophageal strictures, and supraglottic stenosis requiring emergency tracheostomy. Conclusions: Epidermolysis bullosa acquisita may extensively (or predominantly) affect mucosal epithelia in a manner resembling cicatricial pemphigoid. Mucosal disease in these patients is often subclinical, can lead to serious complications, and is best managed using a multidisciplinary approach.

Original languageEnglish (US)
Pages (from-to)954-959
Number of pages6
JournalArchives of Dermatology
Issue number8
StatePublished - Aug 1999

ASJC Scopus subject areas

  • Dermatology


Dive into the research topics of 'Mucosal morbidity in patients with epidermolysis bullosa acquisita'. Together they form a unique fingerprint.

Cite this