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...

Introduction: Anatomic variations of the paranasal sinuses are very common. Paranasal sinus

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...

Epidermolysis bullosa (EB) is a heterogeneous group of rare genetic disorders characterized by marked fragility of the skin and mucous membranes in which vesiculobullous lesions occur in response to trauma, heat or no apparent cause. The recessive form of EB presents the greatest oral alterations including repeated blistering and scar formation leading to limited oral opening, ankyloglossia, to...

Inherited skin fragility disorders comprise a group of disorders, mainly designated as epidermolysis bullosa (EB), that are characterised by mechanical induced blistering and erosions of the skin and mucous membranes caused by mutations of gene coding for protein components of the dermal–epidermal junction zone. Patients with EB experience various degrees of recurrent skin blistering, widesprea...

Chronic leg ulcers occur most frequently in the elderly population as a result of an underlying vascular disease especially chronic venous insufficiency. But it also occurs less commonly in younger people due to other aetiologies, for example, infections, vasculitis, neoplasia or genetic diseases. The following case report presents chronic leg ulcers as a rare cause for the first diagnosis of d...

In dystrophic epidermolysis bullosa, the genetic defect of anchoring fibrils leads to cleavage beneath the basement membrane and its consequent loss. A 46 year-old female patient presented blisters with a pretibial distribution associated with nail dystrophy. Her two children had hyponychia and anonychia, which affected all toe nails and the thumb, forefinger and middle finger. DNA sequencing i...