Epidermolysis

Epidermolysis is a term that describes the separation of the epidermis from the underlying dermis, or the splitting within the epidermal layers, in response to minor trauma. It is a descriptive, not a single disease, and it leads to blistering, erosions, and fragile skin that can be easily damaged by normal touch or movement. The appearance and distribution of lesions can vary with age, underlying cause, and exposure to friction.

The separation can occur at different structural levels in the skin. Intraepidermal epidermolysis refers to cleavage within the epidermal cells, often due to defects in keratin or desmosomal components. Subepidermal epidermolysis involves split at the basement membrane zone, typically associated with defects in hemidesmosomes or basement membrane proteins. The level of cleavage helps classify conditions and guides diagnosis and management.

Causes of epidermolysis range from genetic disorders to acquired conditions. The best-known inherited group is epidermolysis bullosa (EB), a spectrum of disorders caused by mutations in genes encoding skin structural proteins, resulting in varying patterns of fragility and blistering. Other genetic conditions and some autoimmune diseases, burns, radiation, or chemical injuries can also produce epidermolysis. In clinical practice, distinguishing inherited from acquired causes is important for prognosis and family counseling.

Diagnosis typically involves clinical examination, skin biopsy with histology and immunofluorescence mapping to identify the level of epidermal-dermal separation, and, when appropriate, genetic testing. Management focuses on protecting the skin from trauma, meticulous wound care, infection prevention, pain control, and nutritional support. There is no universal cure for most forms of epidermolysis, but multidisciplinary care and emerging therapies aim to reduce morbidity and improve quality of life.