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 oto...

UNLABELLED Epidermolysis bullosa (EB) is a group of skin diseases with different clinical manifestations and varied inheritance patterns. Blisters may appear spontaneously or following minimal trauma to the skin or mucosa. AIM this paper aims to describe the otorhinolaryngological manifestations and esophageal complications related to EB, and the experience in treating patients with esophagea...

Epidermolysis bullosa acquisita is an autoimmune blistering disease of the skin characterized by IgG autoantibodies against type VII collagen. Systemic diseases are often associated with epidermolysis bullosa acquisita, Crohn's disease being the most frequent. This study sought to determine if type VII collagen, the epidermolysis bullosa acquisita autoantigen, was present in normal human colon ...

The objective of this study was to identify and specify the problems of children with epidermolysis bullosa. The questions explored were: (i) What do children with epidermolysis bullosa experience as the most difficult problems; (ii) What is the impact of these problems on their daily life; and (iii) Do these experiences differ between mildly and severely affected children? Qualitative research...

A new keratin-based hydrogel wound dressing was applied to the neck of a patient who was suffering from recessive dystrophic epidermolysis bullosa. A significant improvement was observed in the robustness of skin in this area: reduced propensity to blister and improved healing of blisters. The improvement allowed the cessation of use of secondary dressings for this area. The factors gave a sign...

A patient with epidermolysis bullosa acquisita (EBA) associated with Crohn's disease is presented. The clinical, histological and immunological findings were in keeping with previous reports. However, clinically normal skin and mucosa exhibited deposits of IgG and C3 in the basement-membrane zone. These deposits remained unchanged during the treatment period. It is therefore suggested that immu...

The association between epidermolysis bullosa (EB) and congenital pyloric atresia (CPA) named Carmi Syndrome is rare. We report unusual and morbid complication of gastric perforation resulting in peritonitis in a preterm neonate born with Carmi Syndrome.

Peter B Cserhalmi-Friedman, Maria C Garzon, Edwin Guzman, Amalia Martinez-Mir, Wendy K Chung, Kwame Anyane-Yeboa and Angela M Christiano Department of Dermatology, New York, New York, U.S.A. Department of Genetics and Development, New York, New York, U.S.A. Department of Pediatrics, Division of Clinical Genetics, College of Physicians and Surgeons, Columbia University, New York, New York, U.S.A...

Epidermolysis bullosa (EB) is a group of genetically determined disorders characterized by blistering of the skin and mucosae. There are three major forms--simple, junctional and dystrophic--and each has several varieties. The present case report describes a male child with junctional EB. The aim of the report is to present the dietary situation and the dental status of the child, examples of p...

PURPOSE This study was performed to compare Dental Age (DA) of children with Dystrophic Epidermolysis Bullosa recessiva (DEBr) with the DA of healthy children. METHODS Orthopantomographs (OPG's) of children with DEBr were compared with those of healthy children. Dental maturity was estimated using Dermirjian's method. A total of 48 pairs of OPG's were compared. RESULTS There was a considera...