Epidermolysis bullosa (EB) is a group of heritable mechano-bullous skin diseases classified into three major categories on the basis of the level of tissue separation within the dermal-epidermal basement membrane zone. In the most severe, dystrophic (scarring) forms of EB, blisters form below the cutaneous basement membrane at the level of the anchoring fibrils, which are composed of type VII c...

Epidermolysis bullosa comprises a group of phenotypically different genodermatosis, hereditary or acquired, characterized by skin fragility and subsequent formation of blisters in response to mechanical trauma, and which may also affect mucous membranes. This study aimed to analyze the relation between the nutritional, hematologic, infectious characteristics and the type of epidermolysis bullos...

OBJECTIVES Epidermolysis bullosa is a rare skin disorder characterized by blister formation in response to minor trauma as well as extracutaneous manifestations. Details of the surgical history and technical considerations for performing breast reconstruction in a patient with epidermolysis bullosa are discussed. METHOD The history and details of breast reconstruction in a patient with epider...

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...

Dystrophic epidermolysis bullosa (DEB) pruriginosa (DEB-Pr) is a rare variant of DEB due to COL7A1 dominant and recessive mutations, which is characterized by severe itching and lichenoid or nodular prurigo-like lesions, mainly involving the extremities. Less than 30 patients have been described showing variable disease expression, and frequently, delayed age of onset. We report the clinical an...

Epidermolysis bullosa is a group of inherited disorders that can be both systemic and life-threatening. Standard treatments for the most severe forms of this disorder, typically limited to palliative care, are ineffective in reducing the morbidity and mortality due to complications of the disease. Emerging therapies-such as the use of allogeneic cellular therapy, gene therapy, and protein thera...

Epidermolysis bullosa is a group of disorders whose common primary feature is the formation of blisters following trivial trauma. Recessive dystrophic epidermolysis bullosa (RDEB), a subtype of epidermolysis bullosa, is frequently associated with growth retardation. This growth retardation has been reported to be caused by trophopathy following protein loss through skin lesions. Endocrine disor...

Acquired melanocytic lesions may present unusual clinical features in all forms of hereditary epidermolysis bullosa. These lesions are known as "EB nevi", and often pose a diagnostic challenge for dermatologists given their resemblance - clinically, dermoscopically and histologically - to melanoma. The lesions have been reported in all types of hereditary EB, most of them in childhood. We repor...

OBJECTIVE To report our pediatric experience with amniotic membrane transplantation for ocular surface and forniceal reconstruction. METHODS Retrospective case review of children who underwent superficial keratectomy, symblepharon lysis, and forniceal reconstruction using amniotic membrane transplantation. The underlying diagnosis, visual acuity, level of discomfort at first and last visits, ...