Epidermolytic Ichthyosis

"mosaic epidermolytic ichthyosis--case report.", by Mendes MS, Kouzak SS, Aquino TA, Takano GH, Lima Ade P. f06: epidermolytic hyperkeratosis: parakeratosis, hypergranulosis and vacuolardegeneration of the granular and spinous layer. Hematoxylin and eosin staining...

Summary Epidermolytic ichthyosis specifically refers to a genetic skin disorder that is defined by varying levels of blistering and succeeding reactive scaling of the skin. A palmoplantar keratoderma and/or hair irregularities may exist in some forms of the disorder. Introduction The term epidermolytic ichthyosis was agreed on by the International Ichthyosis Consensus Group in 2009 and replaces the older, puzzling tags of bullous congenital ichthyosiform erythroderma and epidermolytic hyperkeratosis. A number of disorders show epidermolytic hyperkeratosis, consisting of EI, surface epidermolytic ichthyosis bullosa and desmosomal disorders such as McGrath ectodermal dysplasia-skin frailty disorder. Babies with EI might be birthed with red, denuded and blistering skin with noticeable areas of skin enlarging. A generalized erythroderma may be present in some individuals. Skin infections with common microorganisms can be a problem. A palmoplantar keratoderma may exist and can be so extreme as to limit ambulation and hand function. On the other end of the scale, there are individuals that have only minimal blistering in areas subject to friction, or have only a palmoplantar keratoderma.

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