Epidermolytic Hyperkeratosis (EHK)

Epidermolytic Hyperkeratosis (Bullous Congenital Ichthyosiform Erythroderma) is rare, occurring in approximately 1 in 300,000 births.

EHK is characterized by thick, blistering, warty hardening of the skin over most of the body, particularly in the skin creases over the joints. Scales tend to form in parallel rows of spines or ridges. The skin may be fragile and may blister easily following injury. Babies with EHK are usually born with red, blistering and denuded skin with visible skin thickening. Over time there is a gradual decrease in the blistering, but an increase in the severity of the thickness and scaling. A generalized redness of the skin (erythroderma) is present in some individuals. Skin infections and heat intolerance cam be common problems.

Treating EHK can be a challenge. The medications that are used to help remove the excess thickened skin (topical keratolytics or oral retinoids) often remove too much scale, leaving very fragile underlayers exposed. Barrier repair creams, containing ceramides, cholesterol, petrolatum or lanolin, can help along with topical or systemic anti-bacterial agents. Keratolytics and oral retinoids should be used with caution.