It is feasible that the main title of the report Epidermolytic Ichthyosis is not the name you expected. Kindly examine the words detailing to discover the alternative name(s) as well as problem neighborhood(s) covered by this report.
- bullous congenital CIE
- bullous genetic ichthyosiform erythroderma (of Brocq)
Epidermolytic ichthyosis (EI) particularly refers to a genetic skin disorder that is defined by varying levels of blistering and also subsequent responsive scaling of the skin. The underlying histopathology shows mid-epidermal splitting and hyperkeratosis, with each other described as epidermolytic hyperkeratosis (EHK). Depending on the nature of the original mutation, the signs may vary from mild blistering after rubbing to extreme erosions or prevalent warty scaling (“porcupine guy”). A palmoplantar keratoderma (too much callus development on palms and also soles) and/or hair abnormalities might exist in some forms of the disorder.
The term epidermolytic ichthyosis was agreed upon by the International Ichthyosis Consensus Group in 2009 as well as replaces the older, puzzling labels of bullous genetic ichthyosiform erythroderma (Brocq) as well as epidermolytic hyperkeratosis.
A number of disorders show epidermolytic hyperkeratosis, including EI, superficial epidermolytic ichthyosis bullosa as well as desmosomal problems such as McGrath ectodermal dysplasia-skin fragility syndrome. Much confusion has actually arised from use of the term EHK to describe the disorder now called epidermolytic ichthyosis.