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Mucosal Morbidity in Patients With Epidermolysis Bullosa Acquisita
Markham C. Luke, MD, PhD;
Thomas N. Darling, MD, PhD;
Roger Hsu, MD;
Ronald M. Summers, MD, PhD;
Janine A. Smith, MD;
Beth I. Solomon, MS;
Giovana R. Thomas, MD;
Kim B. Yancey, MD
Arch Dermatol. 1999;135:954-959.
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.
From the Dermatology Branch, National Cancer Institute (Drs Luke, Darling, Hsu, and Yancey), the Departments of Diagnostic Radiology (Dr Summers) and Rehabilitation Medicine (Ms Solomon), Clinical Center, the Laboratory of Immunology, National Eye Institute (Dr Smith), and the Head and Neck Surgery Branch, Tumor Biology Section, National Institute of Deafness and Other Communication Disorders (Dr Thomas), National Institutes of Health, Bethesda, Md.
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