Copyright: © 2013 Osada SI, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Successful Treatment of Intravenous Immunoglobulins in a Patient with Intractable Epidermolysis Bullosa Acquisita with Autoant...

This article summarizes recommendations reached following a systematic literature review and expert consensus on the diagnosis and management of cutaneous squamous cell carcinomas in people with epidermolysis bullosa. The guidelines are intended to help inform decision making by clinicians dealing with this complex complication of a devastating disease.

KEYWORDS Epidermolysis bullosa acquisita; Autoimmune subepidermal bullous disease Abstract Background: Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering disease caused by autoantibodies to type VII collagen. The clinical presentation is variable, with skin and mucosal lesions that can cause significant dysfunction. Different treatment options exist, but the results ...

OBJECTIVE To determine whether a series of diseases of the oral mucosa - Sjögren syndrome, ectodermal dysplasia, epidermolysis bullosa and lichen planus - reduce the survival rate of dental implants. MATERIAL AND METHOD A Medline search was carried out using the key words: " Sjögren syndrome ", "ectodermal dysplasia ", "epidermolysis bullosa ", "lichen planus " and "dental implants ", includi...

A human keratinocyte cDNA expression library in bacteriophage lambda gt11 was screened with the purified IgG fraction of serum from a patient with epidermolysis bullosa acquisita, which had a high titer of anti-type VII collagen antibodies. Screening of approximately 3 x 10(5) plaques identified 8 positive clones, the largest one (K-131) being approximately 1.9 kilobases in size. Dideoxynucleot...

Epidermolysis Bullosa Puriginosa is a genetic mechanobullous disease characterized by pruritus, lichenified or nodular prurigo-like lesions, occasional trauma-induced blistering, excoriations, milia, nail dystrophy and albopapuloid lesions that appear at birth or later. Scarring and prurigo are most prominent on the shins. Herein, we report a case with a history of blisters since childhood foll...

A 45-year-old woman with epidermolysis bullosa aquisita is presented. The clinical, histological, and immunopathological features were in keeping with the previous reports of this disease. The patient also had anti-basal cell cytoplasmic antibodies at a significant titer, which is considered an unusual finding associated with this disorder. Treatment with a moderate dose of corticosteroid was e...

In this paper, two patients with severe dysphagia and esophageal stricture secondary to epidermolysis bullosa are presented and discussed. They are siblings of an affected family. Primary resection and anastomosis had been performed previously in both patients, but dysphagia had recurred. We treated these patients with free jejunal graft to the esophagus with excellent results. Both patient...